Photographs of Underwear-Clad Saddam Hussein Published

BAGHDAD, Iraq — British and American newspapers published photos Friday showing an imprisoned Saddam Hussein clad only in his underwear and washing his laundry, prompting an angry U.S. military to launch an investigation and the Red Cross to say the pictures may violate the Geneva Conventions.

Britain’s The Sun and the New York Post said the photos were provided by a U.S. military official they did not identify. The photos not only angered the U.S. military, which issued a condemnation rare for its immediacy.

President Bush said Friday he did not believe the photos would incite further anti-American sentiment in Iraq, which is edging toward open sectarian conflict.

“I don’t think a photo inspires murderers,” Bush said at the White House. “These people are motivated by a vision of the world that is backward and barbaric.”

He added, “I think the insurgency is inspired by their desire to stop the march of freedom.”

Bush was briefed by senior aides Friday morning about the photos’ existence, and he “strongly supports the aggressive and thorough investigation that is already under way” that seeks to find who took them, White House press spokesman Trent Duffy said.

Both The Sun and the Post are controlled by Rupert Murdoch.

Saddam’s chief lawyer, Ziad al-Khasawneh, said his legal team would sue The Sun for publishing what he said represented “an insult to humanity, Arabs and the Iraqi people.”

“It is clear that the pictures were taken inside the prison, which means that American soldiers have leaked the pictures,” he said by telephone from Amman, Jordan. “We will sue the newspaper and everyone who helped in showing these pictures.”

He said the photos were part “of a comprehensive war against the Islamic and Arab nations” that included the abuse at Baghdad’s Abu Ghraib prison and allegations by Newsweek, which were later retracted, about Quran desecration at the U.S. prison in Guantanamo Bay, Cuba.

Iraqis gathered in coffee shops in Baghdad and elsewhere watched as some Arab satellite networks showed the front page of The Sun, with its picture of Saddam standing in his underwear. Other photos show him clothed and seated on a chair doing some washing, sleeping and walking in what is described as his prison yard.

“This is an insult to show the former president in such a condition. Saddam is from the past now, so what is the reason for this? It is bad work from the media. Do they want to degrade the Iraqi people? Or they want to provoke their feelings,” said Abu Barick, a 45-year-old Baghdad businessman.

In northern Kirkuk, Marwan Ibrahim, a 31-year-old civil servant, said the pictures were a “humiliation for a man who in the near past was the leader of Iraq and a top Arab leader in the region.”

Others, however, were not so kind.

“Saddam Hussein and his regime were bloody and practiced mass killing against the people, therefore, whatever happens to Saddam, whether he is photographed naked or washing his clothes, it means nothing to me. That’s the least he deserves,” said Hawre Saliee, a 38-year-old Kurd.

The U.S. military in Baghdad said the photos violated military guidelines “and possibly Geneva Convention guidelines for the humane treatment of detained individuals.”

“The specific issue here is that these images are against (Department of Defense) policy. It’s not the content of the photo that is the issue at hand, but it is the existence or release of the photos,” U.S. military spokesman Staff Sgt. Don Dees said.

He added that the military would question the troops holding Saddam as part of its investigation.

“We take seriously our responsibility to ensure the safety and security of all detainees,” a military statement said.

The military said the source of the photos was not immediately known, but they were believed to have been taken more than a year ago.

The International Committee for the Red Cross, which is responsible for monitoring prisoners of war and detainees, said the photographs violated Saddam’s right to privacy.

“Taking and using photographs of him is clearly forbidden,” ICRC Middle East spokeswoman Dorothea Krimitsas said. U.S. forces are obliged to “preserve the privacy of the detainee.”

Aside from U.S. soldiers, the only others with access to Saddam are his legal team, prosecuting judge Raed Johyee and the ICRC.

Saddam was captured in December 2003 while hiding in a concealed hole in the ground near his hometown of Tikrit, 80 miles north of Baghdad. He is charged with war crimes, but no date has been set for his trial.

It is not the first time there has been an outcry over images of Saddam.

Pictures and video images of Saddam being examined by a medic after his arrest were widely criticized – even by the Vatican. A top Vatican cardinal said at the time that American forces treated the captured Iraqi leader “like a cow.”

Associated Press reporters Bassem Mroue in Baghdad and Jill Lawless in London contributed to this report.

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Malignant Transformation of a Breast Fibroadenoma to Cystosarcoma Phyllodes: Case Report and Review of the Literature

FIBROADHNOMAS HAVE ALWAYS BEEN considered benign tumors of the breast. They are the most frequent benign tumor of the breast after fibrocystic disease. Histologically, they consist of both epithelial and stromal components.1 It is a well-accepted clinical practice for these lesions to be observed after histopathological confirmation.1- 2 Fibroadenomas have not been considered to significantly increase the risk of developing subsequent breast cancer. We present a case of malignant transformation of a fibroadenoma to cystosarcoma phyllodes after 5 years of radiologic stability.

CASE History

A 45-year-old female, G^sub 2^P^sub 0^, was referred to our breast center by her primary care physician in October 2003 for evaluation of a rapidly growing right breast mass at the site of prior fibroadenoma at the 2 o’clock location. She was found to have an 0.9-cm nodule in this area on her baseline mammogram in April 1997 that was occult on sonography (Fig. 1). Stereotactically guided fine-needle aspiration (FNA) and core biopsy of this nodule revealed a fibroadenoma (Fig. 2). The nodule remained radiographically stable from April 1997 to June 2002.

On physical examination, the patient was found to have a 3-cm firm mobile mass in the 2 o’clock position of the right breast without evidence of any lymphadenopathy. Mammogram in October 2003 showed that the palpable mass corresponded with a lobulated dense, partially circumscribed noncalcified 4 3-cm mass, which represented a major interval change (Fig. 3). A focused right breast ultrasound corresponded with a lobulated heterogenous solid mass at the 2 o’clock axis, 2 cm from the nipple and 1 cm deep to the skin, measuring 2.8 2.0 2.7 cm (Fig. 4). This corresponded to the site of prior 0.9-cm nodule.

A fine-needle aspiration in October 2003 was consistent with atypical cells, with mixed features of cellular biphasic tumor and cystic papillary changes. The patient underwent wide local excision. Pathology revealed malignant cystosarcoma phyllodes, 2.5 cm in maximum diameter, with 1-cm margins (Figures 5-7).

Discussion

Most women diagnosed with fibroadenomas are treated with observation. Fibroadenomas are typically diagnosed in young women with the average age of patients being 20-30 years old.1 Due to the fact that it is acceptable to observe fibroadenomas, it is crucial to identify risk factors for malignant transformation of these lesions.

Cystosarcoma phyllodes, similar to fibroadenoma, typically present as a firm, seldom painful, smooth, well-circumscribed and mobile mass.1,3 It demonstrates continuous growth, but it may also grow rapidly resulting in shiny stretched skin with visible underlying veins. As a result of this, the skin can ulcerate due to ischemia. Differentiation between malignant versus benign cystosarcoma phyllodes should not be based on the presenting symptoms such as pain, retraction of the nipple and skin fixation, as they can occur in both entities.3

It is quite challenging to differentiate fibroadenoma and cystosarcoma phyllodes by either mammography or sonography.1,3 A confirmed diagnosis occurs in only 32 per cent of mammograms.3 In approximately 10 per cent of cases, cystosarcoma phyllodes are seen mammographically in the absence of a palpable mass. Sonography may demonstrate small cystic areas within an otherwise benign appearing mass, which would require a biopsy.4

The average age of patients with cystosarcoma phyllodes is 40 years.1,4 Seventy per cent of these tumors occur between ages 30 and 55 years. However, they have been reported in girls as young as age 10 and into the ninth decade. The incidence of these tumors in women younger than age 25 may approach that of breast carcinoma. They occur rarely in men.4 Although a cystosarcoma phyllodes tumor contains epithelial and stromal components with similar histological characteristics to fibroadenoma, the stroma in phyllodes has increased cellularity, pleomorphism, nuclear atypia, and mitotic figures.1, 3-5 When compared to fibroadenomas, the natural history of cystosarcoma phyllodes is quite different. Fibroadenomas tend to grow slowly and often reach a maximum diameter of 2-3 cm in size. A significant percentage of fibroadenomas regress spontaneously, whereas cystosarcoma phyllodes tumors grow rapidly and can attain sizes of 10 cm or more.1

The diagnosis of cystosarcoma phyllodes tumor cytologically is challenging, with significant overlap with fibroadenoma. Large size, low epithelial/stromal ratio, epithelial atypia, columnar stromal cells with visible cytoplasm, and stromal giant cells favors a phyllodes tumor rather than a fibroadenoma.6 The lesion can be categorized from benign to borderline to malignant based on whether there are infiltrative borders, the degree of stromal overgrowth, the presence of cellular atypia, and the proportion of mitotic figures.4, 7, 8 According to Norris and Taylor, 0-4 mitoses/10 hpf with minimal atypia was classified as benign, 5-9 mitoses/10 hpf with moderate atypia as borderline, and >10 mitoses/10 hpf with marked atypia as malignant.3 Azzopardi and Salvador! used the same histological classification to categorize these lesions.9 Fine- needle aspiration by an experienced cytopathologist is a highly reliable tool in diagnosing cystosarcoma phyllodes tumors10, 11 with accuracy rate of 92.8 per cent.10 To avoid diagnostic error, multiple-site aspiration of the mass is recommended.3 However, only a “positive” biopsy, revealing the features of a malignant phyllodes tumor or the benign features of a fibroadenoma can be relied on. All other findings would require excisional biopsy.4

Cystosarcoma phyllodes account for 0.3-0.9 per cent of all breast tumors1 3-5, 7, 9 and only 2-3 per cent of all mammary fibroepithelial neoplasms.3, 7 The incidence rate is 1 in 100,000 with an overall risk of malignancy of 2.1 per 1 million women. There are no predisposing factors, and the etiology is unknown. One study reported Latin women at higher risk than other ethnic groups. There has been no documented relationship between menopausal status or use of oral contraceptives and development of these tumors. A strong correlation between development of these tumors and nulliparity was demonstrated in one study, in which all the patients with malignant cystosarcoma phyllodes tumor were nulliparous.3

The right breast is the more common site for this tumor. In one study, 62.5 per cent of tumors were in the right breast, with the remaining 37.5 per cent in the left breast. Bilaterality was as high as 30 per cent in one study. Of these tumors, 32-36 per cent occur in the upper outer quadrant, 14-17 per cent in the upper inner quadrant, 11-25 per cent in the lower outer quadrant, and 6-8 per cent in the lower inner quadrant. In the same study, 37 per cent of the tumors presented in multiple sites.3

FIG. 1. Initial mammogram demonstrating an 0.9-cm nodule, biopsy proven to be a fibroadenoma.

These tumors range from 1 to 41 cm, with median size of 5 cm. In one study, 73 per cent of benign phyllodes tumors were 7 cm.3 The term cystosarcoma phyllodes was first described and coined by Johannes Muller in 1838, which he clearly stated to be a benign tumor.3-5, 7 They are commonly benign, considering this fact, the World Health Organization coined the term “phyllodes tumor” in 1981.3 However, malignant types exist and can metastasize.3, 12 Lee and Pack reported the first case of metastatic phyllodes tumor in 1931.3,5

High-grade malignant phyllodes tumor (MPT) forms approximately 25- 35 per cent of all phyllodes tumors.4, 9 Approximately one-third to one-half of the malignant tumors will metastasize.4 However, the overall metastatic rate is

FIG. 2. Core biopsy of the breast lesion performed in 1997. Histologic features are those of a fibroadenoma. Note the hypocellular stroma (H&E, 20).

FIG. 3. Mammogram in 2003, demonstrating 4 3-cm mass corresponding to the palpable mass.

FIG. 4. Focused breast ultrasound in 2003, demonstrating a tabulated heterogenous solid mass, measuring 2.8 2.0 2.7 cm.

FIG. 5. Biopsy of the same lesion performed in 2003. Note the complex epithelium-lined spaces characteristic of a phyllodes tumor (H&E, 20).

Haagensen was one of the early advocates for wide excision instead of mastectomy.5 Wide local excision with 1-2 cm margins remains the mainstay of treatrnent.3-5, 8, 9, 12, 14 Simple mastectomy is preferred for larger tumors, in cases where appropriate margin clearance can not be achieved or for repeated local recurrencesdespite adequate margins.4, 12 Due to the fact that these tumors do not metastasize via lymphatic drainage, sentinel lymph node biopsy or axillary lymph node dissection is not recommended unless they are clearly involved with tumor.3-5, 7, 9 Palpable axillary lymph nodes is reported in 20 per cent of patients with phyllodes tumor5; however, only 5 per cent of all patients with palpable nodes have histological proof of malignancy,3, 5 and less than 1 per cent of all patients with MPT had positive nodes.9 Because in the setting of even malignant phyllodes tumors palpable lymphadenopathy is almost always reactive3, 4, 9 from tumor necrosis or secondary infection of ulcerating lesions,9 the presence of metastatic disease in the nodes should be confirmed prior to nodal dissection.3, 4

FIG. 6. Malignant phyllodes tumor. High-power magnification showing a cellular stroma consisting of spindle cells with nuclear atypia and abnormal mitoses (H&E, 400).

FIG. 7. Malignant phyllodes tumor. Residual area with hypocellular stroma and pericanalicular pattern of fibroadenoma (H&E, 40).

The role of adjuvant chemotherapy or radiation therapy is not well established due to lack of randomized trials secondary to rarity of this tumor.3, 5, 12, 15 Currently, there is no consensus regarding the use of adjuvant treatment.3, 8, 15 Although it has been shown that 20-40 per cent of phyllodes tumors are estrogen receptor positive and all exhibit the presence of progesterone receptors,3, 9 hormone therapy has no established role in their treatment.3-5, 9 However, in selected cases radiotherapy may be useful,3, 8, 13 such as inability to attain negative margins.14 There is anecdotal evidence suggesting a possible role for radiotherapy for borderline or malignant phyllodes tumors in the setting of breast conservation and when treated with mastectomy when the local failure risk is high. Chaney et al. reported on their experience with adjuvant radiation therapy for 8 patients with localized phyllodes tumors (2 benign, 1 borderline, 5 malignant; size 3.5-15 cm). With follow up of 22-84 months, they reported no local or distant failures. They concluded that adjuvant radiation therapy may be useful at doses of 50-60 Gy for patients with positive, 10 cm, or following resection of recurrent disease.8 Pandey et al. reported that adjuvant RT is thought to decrease local recurrence (LR) with no significant effect on survival.7 Furthermore, adjuvant radiation therapy should be considered in patients demonstrating stromal overgrowth in tumors >5 cm.3 The role of chemotherapy seems to be limited to the treatment of metastasis and for palliation of unresectable local recurrence.7

Local recurrence occurs 5-15 per cent for benign tumors and 20- 30 per cent for malignant cases.3, 14 Kapiris et al.9 reported the LR of MPT at 44 per cent and DM at 27 per cent. They concluded that both tumor size and margin status are important factors in local recurrence and distant metastasis. They found a 7-fold increase in LR for tumors greater than 10 cm and a 4-fold increase in LR when the surgical margins were involved. In their study, they also demonstrated LR to be an important predictor of future metastatic disease, with 85 per cent of patients who ultimately developed metastases, had suffered a previous LR.9 August et al. suggested that if radiation therapy is used after wide local excision of high risk lesions (tumor >5 cm, presence of stromal overgrowth, >10 mitoses/ hpf, infiltrating margins), for all comers local recurrence would likely be avoided in 90 per cent or more of patients.4 Chaney et al. reported stromal overgrowth as the strongest predictor of distant metastasis and ultimate outcome. Due to this fact, they feel that patients with stomal overgrowth feature, and specifically those with tumors >5 cm, should be considered for adjuvant chemotherapy.14

Pandey et al. reported the 5-year overall survival for patients with malignant phyllodes tumors at 74.2 per cent and the 5-year disease-free survival at 59.6 per cent.7 Chaney et al. reported 5- year and 10-year survival rates of 82 per cent and 42 per cent, respeclively.14 Pandey et al. and Shabahang et al. reported that the margin of surgical excision was the only independent prognostic factor for long-term survival.7- 16 However, it was felt that patients with larger tumors, >5 cm, had increased hazard, whereas those undergoing radiotherapy, married women, and age >35 showed a decreased hazard.7 Mortality is secondary to direct extension of local recurrences into the chest or from metastasis to lung, bone, or both.5

In a study by Noguchi et al.1 investigating the clonal analysis of fibroadenoma and phyllodes tumor it was shown that in fibroadenoma both epithelial cells and stromal cells were polyclonal. However, phyllodes tumor consists of polyclonal epithelial cells and monoclonal stromal cells. Therefore, phyllodes tumor can be regarded as stromal cells neoplasm. Because phyllodes tumor is often preceded or accompanied by fibroadenoma and these two entities have some histological similarity, they speculated that phyllodes tumor begins as fibroadenoma and subsequently a single stromal cell undergoes mutation and develops into a phyllodes tumor composed mainly of monoclonal stomal cells and partially of polyclonal epithelial cells. These data may prove to be clinically useful to distinguish fibroadenoma from phyllodes tumor by clonal analysis on small DNA samples obtained by FNA cytology.1

In a 1995 study, Noguchi et al.2 reported three cases of fibroadenoma that were diagnosed by excisional biopsy that recurred as benign phyllodes. Clonal analysis showed that all three fibroadenomas were monoclonal in origin. These results were inconsistent with their previous findings that fibroadenoma was polyclonal. Hence, it was suggested that from the clonal point of view there are two types of fibroadenoma, polyclonal and monoclonal, but they could not be differentiated histologically. It is speculated that the incidence of monoclonal fibroadenoma is quite low, therefore most of the usual fibroadenomas that can be cured by enucleation are polyclonal in origin and without tendency to recur locally. The percentage of monoclonal fibroadenomas against total fibroadenomas remains to be determined. Furthermore, the recurrent phyllodes tumors showed inactivation of the X chromosome-linked androgen receptor (AR) gene, which was also demonstrated in the primary fibroadenoma in each case. Hence, based on these results, they speculated that the recurrent phyllodes tumors were true recurrences of the primary fibroadenoma. The assumption was that the recurrence was due to residual tumor secondary to inadequate excision of the initial fibroadenoma. The authors therefore believe that fibroadenoma can progress to phyllodes tumor. Thus, they speculated that monoclonal fibroadenoma starts as a polyclonal fibroadenoma and due to a stromal cell line undergoing mutation, it forms a monoclonal fibroadenoma, which subsequently can progress to phyllodes tumor. Therefore, it was suggested that monoclonal fibroadenoma should be treated like phyllodes tumor with wide local excision.2

Follow-up after definitive surgery for malignant phyllodes tumor consists of biannual physical exam for 5 years, then annual, baseline unilateral mammogram 3 months postexcision with/without radiation if breast was conserved, then annual bilateral screening mammography. Biannual CT of the chest should be performed for 2-5 years for high-risk lesions.4

Conclusion

Based on the data from the literature and the case presented in this paper, it can be concluded that there are monoclonal fibroadenomas that can progress to phyllodes tumor. Therefore, it is important to differentiate between monoclonal and polyclonal fibroadenoma,as the former is treated surgically and the later is monitored clinically. Because the PCR method for clonal analysis can be performed on FNA sample, this distinction can be achieved without surgery.2 However, because performing clonal analysis on all fibroadenomas is time consuming and not cost effective, it may be a potentially valuable tool in evaluating rapidly growing fibroadenomas.

REFERENCES

1. Noguchi S, Motomura K, Inaji H, et al. Clonal analysis of fibroadenoma and phyllodes tumor of the breast. Cancer Res 1993; 53:4071-4.

2. Noguchi S, Yokouchi H, Aihara T, et al. Progression of fibroadenoma to phyllodes tumor demonstrated by clonal analysis. Cancer 1995;76:1779-85.

3. Guerrero MA, Ballard BR, Grau AM. Malignant phyllodes tumor of the breast: review of the literature and case report of stromal overgrowth. Surg Oncol 2003; 12:27-37.

4. August DA, Kearney T. Cystosarcoma phyllodes: mastectomy, lumpeetomy, or lumpectomy plus irradiation. Surg Oncol 2000;9:49- 52.

5. Geisler DP, Boyle MJ, Malnar KF, et al. Phyllodes tumors of the breast: a review of 32 cases. Am Surg 2000;66:360-6.

6. Tse GM, Ma TK, Pang LM, dieting H. Fine needle aspiration cytologie features of mammary phyllodes tumors. Acta Cytol 2002;46:855-63.

7. Pandey M, Mathew A, Kattoor J, et al. Malignant phyllodes tumor. Breast J 2001;7:411-6.

8. Chaney AW, Pollack A, McNeese M, Zagars GK. Adjuvant radiotherapy for phyllodes tumor of breast. Radiat Oncol Investig 1998;6:264-7.

9. Kapiris I, Nasiri N, A’Hern R, Healy V, Gui GP. Outcome and predictive factors of local recurrence and distant metastases following primary surgical treatment of high-grade malignant phyllodes tumours of the breast. Eur J Surg Oncol 2001;27:723-30.

10. Jayaram G, Sthaneshwar P. Fine-needle aspiration cytology of phyllodes tumors. Diagn Cytopathol 2002;26:222-7.

11. Scolyer RA, McKenzie PR, Achmed D, Lee CS. Can phyllodes tumours of the breast be distinguished from fibroadenomas using fine needle aspiration cytology? Pathology 2001;33:437-43.

12. Shabbir J, O’Sullivan JB, Mahmood S, Byrnes G. Phyllodes tumor of breast. J CollPhysicians Surg Pak 2003; 13:170-1.

13. Harada S, Fujiwara H, Hisatsugu T, Sugihara H. Malignant cystosarcoma phyllodes with lymph node metastasis; case report. Jpn J Surg 1987;17:174-7.

14. Chaney AW, Pollack A, McNeese MD, et al. Primary treatment of cystosarcoma phyllodes of the breast. Cancer 2000;89:1502-11.

15. Khan SA, Badve S. Phyllodes tumors of the breast. Curr Treat Options Oncol 2001;2:139-47.

16. Shabahang M, Franceschi D, Sundaram M, et al. Surgical management of primary breast sarcoma. Am Surg 2002;68:673-7.

EDNA K. VALDES, M.D., SUSAN K. BOOLBOL, M.D., JEAN-MARC COHEN, M.D., SHELDON M. FELDMAN, M.D., F.A.C.S.

From the Louis Venet Comprehensive Breast Service, Beth Israel Medical Center, New York, New York

Address correspondence and reprint requests to Sheldon M. Feldman, M.D., F.A.C.S., Phillips Ambulatory Care Center, 10 East Union Square, Suite 4E, New York, NY 10003.

Copyright The Southeastern Surgical Congress Apr 2005

Mucinous Cystadenoma of the Pancreas Associated With Acute Pancreatitis and Concurrent Pancreatic Pseudocyst

We report an unusual occurrence of a recurrent pancreatic pseudocyst caused by an underlying mucinous cystadenoma of the distal pancreas. A 54-year old female was admitted for acute pancreatitis. Her only risk factors included the use of hydrochlorothiazide and two or three glasses of wine daily. Abdominal computed tomography (CT) done a week after onset of her symptoms showed a 5-cm cystic lesion in the tail of the pancreas suspected to be a pseudocyst. Her symptoms subsequently resolved. One month later, she had another episode of pancreatitis and an abdominal CT showed an 11 16 cm pseudocyst along with the previously mentioned cystic lesion. Approximately 6 weeks after her initial presentation, she was taken to the operating room for an exploratory laparotomy and cyst gastrostomy for a symptomatic pseudocyst. An intraoperative frozen section of the cyst wall showed a fibrous wall with acute and chronic inflammation without an epithelial lining. Six weeks after her cyst gastrostomy, she returned with abdominal pain, early satiety, and anorexia. Abdominal CT showed reaccumulation of fluid within the pseudocyst and endoscopie retrograde cholangiopancreatography (ERCP) revealed a normal caliber pancreatic duct with an abrupt cutoff at the distal duct. She underwent exploratory laparotomy with drainage of 3 L of fluid from the pancreatic pseudocyst. After gaining access to the lesser sac, a 6-cm cystic lesion was identified in the tail of the pancreas. She underwent a distal pancreatectomy and splenectomy. The intraoperative and final pathology confirmed the presence of a benign mucinous cystadenoma. The patient had an uneventful recovery, began to tolerate oral intake, and was discharged 7 days after surgery. The differentiation between a pancreatic pseudocyst and benign cystic neoplasms of the pancreas is crucial to determine treatment options. Cystic neoplasms of the pancreas, whether mucinous or serous, have the potential to harbor malignancy, and resection is recommended.

THE CLINICAL DIFFERENTIATION BETWEEN benign cystic neoplasms of the pancreas and pancreatic pseudocysts can be difficult, but this is important because the treatment of these entities is quite different. Pancreatic pseudocysts are localized collections of pancreatic secretions in a cystic structure that lack an epithelial lining and occur as a result of surrounding tissues walling off a contained pancreatic duct disruption. The management of pseudocysts depends on the clinical setting, but they can often be managed non- operatively. Cystic neoplasms of the pancreas, whether mucinous or serous, have the potential to harbor malignancy, and resection is recommended for low-risk patients. Different imaging modalities, including computed tomography (CT), magnetic resonance imaging (MRI), and endoscopie ultrasound (EUS), can be employed to further differentiate these pancreatic lesions and guide decisions for clinical management.

We report the unusual occurrence of a patient with recurrent pancreatitis and a pancreatic pseudocyst caused by the underlying presence of a benign mucinous cystadenoma of the distal pancreas. There is one previous case report of a mucinous cystadenoma causing acute pancreatitis1 and another case report of the coexistence of a pseudocyst with a mucinous cystadenoma.2 We present the first case report of a mucinous cystadenoma of the pancreas associated with acute pancreatitis and concurrent pancreatic pseudocyst.

Case Report

A 54-year-old white female was admitted for acute pancreatitis after a 3-week history of midepigastric pain, nausea, early satiety, and episodic diarrhea. Her past medical history was significant for benign hypertension, which was currently controlled with hydrochlorothiazide, and she had undergone a laparoscopic cholecystectomy and hysterectomy in the past. She did report drinking two to three glasses of wine per night for approximately 5 years. The admission laboratory tests showed normal liver function tests, normal white blood cell count, but her amylase and lipase were 243 U/L (normal range, 27-137 U/L) and 2447 U/L (normal range,

However, she returned 3 days after discharge with recurrent midepigastric pain, nausea, and vomiting, and continued early satiety. Contrasted abdominal CT showed a 4-5 cm cystic mass in the midbody/tail of the pancreas with an anterior rim of normal appearing pancreas (Fig. 1). Approximately 1 week later, she was referred to our institution with her outside CT scans and was asymptomatic. At this point, it was believed that she had an acute pancreatic pseudocyst; however, a cystic neoplasm of the pancreas was still in our differential diagnosis. It was recommended that she return in 6 weeks for a follow-up CT scan, abstain from alcohol, discontinue her diuretic use, and eat a low-fat diet.

One month after her last admission, she returned to the emergency room with a 1 -week history of increasing midepigastric pain with radiation to her back and worsening of her early satiety. Her laboratory tests in the emergency room again showed elevated amylase and lipase of 598 U/L and 3036 U/L, respectively. Contrasted CT of the abdomen and pelvis showed the aforementioned 4-5 cm cystic lesion in the midbody/tail of the pancreas, but there was interval development of an additional larger cystic lesion measuring 11 16 cm at its largest diameter. This larger cystic lesion occupied the majority of the anterior abdomen, displacing the stomach, colon, and small bowel, and extended into the pelvis. There was significant compression of the body and tail of the pancreas adjacent to these cysts, but otherwise the pancreas appeared normal without ductal dilatation (Figs. 2, 3, and 4). The working diagnosis was a complex pseudocyst, but the original lesion was still distinctly seen.

FIG. 1. CT scan of the abdomen showing a 4-5 cm cystic mass in the body/tail of the pancreas.

FIG. 2. CT scan of the abdomen showing the previously seen cystic mass (Fig. 1) in the pancreas and interval development of additional large cystic lesions.

She returned to our institution with continued symptoms from this enlarging cystic lesion and was eager for operative intervention. Approximately 6 weeks after her initial presentation, she underwent an exploratory laparotomy and cyst gastrostomy draining 1.8 L of fluid from her pancreatic pseudocyst. An intraoperative frozen section of the cyst wall showed a fibrous wall with acute and chronic inflammation without an epithelial lining (Fig. 5) compatible with a pseudocyst. She was discharged from the hospital 3 days later.

One month after her cyst gastrostomy, she returned to the emergency room with a 2-week history of intermittent nausea, recurrent early satiety, and increasing abdominal distention. The day of presentation she experienced an acute onset of severe midepigstric pain with nausea and inability to tolerate oral intake. In the emergency room, her vital signs were normal, her laboratory values were normal, with the exception of an amylase and lipase of 428 U/L and 794 U/L, respectively. Her physical exam was significant for tenderness to palpation in the midepigastric area with obvious abdominal distention. Contrasted CT scan of the abdomen and pelvis showed reaccumulation of fluid within the pseudocyst cavity (Figs. 6, 7, and 8). Endoscopie retrograde cholangiopancreatography (ERCP) revealed a normal caliber common bile duct, normal common hepatic ducts with a normal intrahepatic biliary tree, and a normal cystic duct stump (Fig. 9). The pancreatogram showed a normal caliber main pancreatic duct out to the junction of the body and tail. However at this level, there was an abrupt cutoff suggestive of a high-grade stricture (Figs. 10 and 11). There was no evidence of communication between the main pancreatic duct and the patient’s psuedocyst.

With evidence of a high-grade distal pancreatic ductal stricture and possible distal ductal disruption, we decided to perform an exploratory laparotomy, distal pancreatectomy, and splenectomy. At surgery, 3 L of fluid were drained from the pancreatic pseudocyst, which occupied most of the abdominal cavity. Intraoperative frozen section of the cyst wall was consistent with a pancreatic pseudocyst. After access to the lesser sac was obtained through a small cystotomy, the head and body of the pancreas appeared normal. However, there were dense adhesions and inflammation surrounding a 6- cm cystic lesion in the tail of the pancreas. After removal of the distal pancreas and spleen, intraoperative frozen sections of the cystic lesion in the tail were consitent with a mucinous cystadenoma. The final pathology confirmed the presence of a mucinous cystadenoma with its characteristic features: a mucin- producing columnar epithelium lining the cyst wall, underlying ovarian-type stromal tissue, and no evidence of dysplasia (Fig. 12). The patient had an uneventful recovery, began to tolerate oral intake, and was discharged 7 days after surgery.

FIG. 3. CT scan of the abdomen showing the previously seen cystic mass (Fig. 1) in the pancreas and interval development of additional large cystic lesions.

FIG. 4. CT sc\an of the abdomen showing the previously seen cystic mass (Fig. 1) in the pancreas and interval development of additional large cystic lesions.

FIG. 5. Histologic features of a pancreatic pseudocyst: fibrous wall with acute and chronic inflammation without an epithelial lining.

FIG. 6. CT scan of the abdomen 1 month after a cyst gastrostomy demonstrating reaccumulation of fluid within the pseudocyst cavity.

Discussion

Mucinous cystadenomas are a benign type of mucinous cystic neoplasm of the pancreas (MCNs). Patients with mucinous cystadenomas have a mean presentation of 48 years with a distinct female predominance as high as 9:1 in some series.3-4 These cystic tumors are typically large (50% are greater than 5 cm) and most (greater than 50%) are located in the distal body and tail of the pancreas. Patients most often present with nonspecific abdominal pain, nausea, vomiting, and weight loss.

In published series, very few patients with mucinous cystadenomas present with acute pancreatitis nor are they complicated by the development of a pancreatic pseudocyst.3-4 There is only one case in the literature that attributes the development of acute pancreatitis to a mucinous cystadenoma.1 Likewise, there is only one case that describes the coexistence of a pseudocyst and a mucinous cystadenoma.2 There are no reported cases of a mucinous cystadenoma causing pancreatitis and recurrent pancreatic pseudocysts.

FIG. 7. CT scan of the abdomen 1 month after a cyst gastrostomy demonstrating reaccumulation of fluid within the pseudocyst cavity.

FIG. 8. CT scan of the abdomen 1 month after a cyst gastrostomy demonstrating reaccumulation of fluid within the pseudocyst cavity.

FIG. 9. ERCP showing a normal biliary ductal system and a cystic duct stump.

FIG. 10. Pancreatogram revealing a high-grade stricture of the pancreatic duct at the junction of the body/tail.

Many imaging modalities can be useful for differentiating between pancreatic pseudocysts and mucinous cystadenomas. Abdominal computeed tomography is now one of the most commonly used diagnostic tools for the differentiation between pseudocysts and cystic tumors. Rattner et al. suggested that computed tomography showing solid components in the wall of the cyst is highly suggestive of a neoplastic process.5 Most pseudocysts are single and have few solid components, septae or loculations, or calcifications in the cyst wall. In our case, the two cystic lesions had none of these differentiating features to aid our diagnosis. In a previously reported case, magnetic resonance imaging easily differentiated a pseudocyst from a mucinous cystadenoma.2 T1-weighted, T2-weighted, and gadolinium-enhanced images may delineate loculations, septae, and further define the surrounding tissue in order to differentiate the cystic lesions.

Endoscopic ultrasound can also play a role in further characterizing cystic pancreatic lesions by providing additional morphologic detail, but it can also allow for sampling of fluid from the lesion for measurement of enzymes, viscosity, measurement of tumor markers, and cytologie examination. The literature is controversial with respect to the ability of EUS to differentiate cystic lesions of the pancreas. In a prospective study by Song et al., they concluded that EUS-determined imaging criteria were useful in delineating pancreatic pseudocysts from cystic neoplasms. In their experience, pseudocysts contained echogenic debris and demonstrated parenchymal changes more often than cystic neoplasm. In addition, internal septae were seen with EUS more frequently in cystic neoplasms.6 However, Ahmad et al. also evaluated the utility of EUS in differentiating benign from malignant cystic lesions of the pancreas. They concluded that endosonographic features alone were not reliable in differentiating these lesions.7

FIG. 11. Pancreatogram revealing a high-grade stricture of the pancreatic duct at the junction of the body/tail.

Cytologic analysis and evaluation of various tumor markers within cyst fluid may be used for cyst diagnosis. Mucin and mucinous cells are characteristic of mucinous cystic neoplasm, glycogen-staining cells are seen in serous cystadenomas, and inflammatory cells and histiocytes are associated with pseudocysts. A high concentration of amylase in the cyst fluid is diagnostic of a pancreatic psuedocyst. Similarly, a high carcinoembryonic antigen (CEA) is indicative of a mucinous cystadenoma and a CEA of greater than 400 is potentially predictive of malignancy.8

The importance of distinguishing cystic neoplasms (benign and malignant) from pseudocysts is essential to treatment of these entities. Pseudocysts may be managed multiple ways depending on symptomatology, size, and location. Nonoperative management and surveillance is appropriate in patients who are not symptomatic from their lesion. Pseudocysts that persist, cause symptoms, or become infected need to be addressed by an operative or nonoperative approach. Nonoperative drainage can be performed percuteaneously or endoscopically. Operative therapy provides internal drainage via a cystojejunostomy, cystogastrostomy, or a cystoduodenostomy, depending on the location and characteristics of the lesion. Because serous cystic neoplasms are virtually always benign, resection should probably be reserved for those patients who are symptomatic and when differentiation from a mucinous neoplasm cannot be made confidently. In contrast to serous cystic neoplasms, mucinous cystic neoplasms should be considered premalignant and be resected.

Benign and malignant cystic neoplasms are simply differentiated based on pathology. Any cystic neoplasm exhibiting invasive components is considered a malignant cystadenocarcinoma. For patients with benign serous or mucinous cystadenomas, complete resection is curative. Patients with resected mucinous cystadenocarcinomas typically live much longer than those with resected ductal adenocarcinoma, with an approximate 50 per cent 5- year survival.9

In conclusion, the current case illustrates the difficulty, yet the importance, in definitively distinguishing pancreatic psuedocysts from cystic neoplasms of the pancreas. There are multiple strategies in the management of pancreatic pseudocysts, both operative and nonoperative, but these would be inappropriate for cystic pancreatic neoplasms, benign and malignant. Imaging modalities, cyst fluid analysis, and cytologic fluid analysis help distinguish cystic lesions of the pancreas.

FIG. 12. Histologic features of a mucinous cystadenoma: mucin- producing columnar epithelium lining the cyst wall with underlying ovarian-type stromal tissue.

REFERENCES

1. Sperti C, Pasquali C, Davoli C, et al. Mucinous cystadenoma of the pancreas as the cause of acute pancreatitis. Hepatogastro enterology 1998;45:2421-4.

2. Hsieh CH, Tseng JH, Huang SF. Co-existence of a huge pseudocyst and mutinous cystadenoma: report of a case and the value of magnetic resonance imaging for differential diagnosis. Eur J Gastroenterol Hepatol 2002;14:191-4.

3. Sarr MG, Carpenter HA, Prabhakar LP, et al. Clinical and pathologic correlation of 84 mutinous cystic neoplasms of the pancreas-can one reliably differentiate benign from malignant (or or premalignant) neoplasms? Ann Surg 2000;231:205-12.

4. Warshaw AL, Compton CC, Lewandrowski K, et al. Cystic tumors of the pancreas: New clinical, radiologie, and pathologic observations in 67 patients. Ann Surg 1990;212:432-45.

5. Rattner DW, Castillo CF, Warshaw AL. Cystic pancreatic neoplasms. Ann Oncol 1999;10(suppl 4):s107-10.

6. Song MH, Lee SS, Park JS, et al. The role of endoscopic ultrasonography in pancreatic cystic lesions. Gastrointes Endosc 2002;55:AB249.

7. Ahmad NA, Kochman ML, Lewis JD, Ginsberg GC. Can EUS alone differentiate between malignant and benign cystic lesions in the pancreas? Am J Gastroenterol 2001;96:295-300.

8. Fernandez C, Warshaw AL. Cystic neoplasms of the pancreas. Pancreatol 2001;1:641-7.

9. Wilentz RE, Albores-Saavedra J, Zahurak N, et al. Pathologic examination accurately predicts prognosis in mutinous cystic neoplasms of the pancreas. Am J Surg Pathol 1999;23:1320-7.

ROBERT T. RUSSELL, M.D., KENNETH W. SHARP, M.D.

From Vanderbilt University Medical Center, Nashville, Tenneesee

Presented at the Annual Scientific Meeting and Postgraduate Course Program, Southeastern Surgical Congress, New Orleans, LA, February 11-15, 2005.

Address correspondence and reprint requests to Kenneth W. Sharp, M.D., Vanderbilt University Medical Center, Room D, 5203 Medical Center North, Nashville, TN 37232-2577.

Copyright The Southeastern Surgical Congress Apr 2005

Allegations of Negligence Follow Doctor

SYDNEY, Australia (AP) — After Des Bramich’s sternum was crushed when a camper he was working under fell on him, his family thought the worst was over when he recovered enough to make jokes after being rushed to a hospital emergency room.

But complications soon set in, and the director of surgery at the rural Australian hospital decided that it was necessary to drain excess fluid from Bramich’s chest using a procedure that required a large needle to be pushed into a sac surrounding his heart.

Bramich died the day after the treatment. His son, Mark, said he later learned from hospital staff that the surgeon, Jayant Patel, had to thrust in the needle up to 50 times before finally getting the procedure right.

It’s not clear if the procedure contributed directly to Bramich’s death, but his family is among about 100 former patients or their relatives who have filed lawsuits or criminal complaints against Patel for alleged negligence. Homicide detectives are investigating some of the claims.

Medical reports and interviews by The Associated Press with the families of former patients and Patel’s co-workers indicate a pattern of alleged malpractice that has trailed the Indian-born doctor for years – from the United States to Australia and now into hiding, and earned him the media sobriquet “Dr. Death.”

The scandal has triggered reviews of medical licensing procedures in Australia and Oregon, where Patel was disciplined for gross negligence in 2000 and forced to surrender his medical license issued by New York State, according to officials and documents.

On Monday, officials in Queensland state will open an inquiry into Patel’s record at the Bundaberg Base Hospital, where former colleagues have linked him to the death or serious injury of at least 14 patients, including Bramich.

An initial investigation indicated the scandal could be much broader. A report by the state health department, Queensland Health, found this week that 110 of 1,202 patients Patel treated in two years either died or had to be transferred elsewhere for further treatment – a much higher ratio than normal, doctors say.

The Queensland inquiry will try to determine why Patel was allowed to practice in Australia despite a history of botched operations.

Bramich was helping a friend do some repairs on the camper last July when it fell, his son Mark told the AP by telephone from his home near Bundaberg, about 186 miles north of Queensland’s capital, Brisbane.

He was rushed to the hospital in critical condition, though he was quickly stabilized and moved from intensive care to a regular ward. Nurses arranged for him to be transferred to a Brisbane hospital with a cardio-thoracic surgeon, said Mark Bramich and a nurse who spoke on condition of anonymity.

But Patel slowed the transfer and insisted on performing the fluid draining procedure first, said the nurse, who is to testify to the inquiry.

It was then that Bramich’s family noticed signals that something was wrong, Mark Bramich said.

“The nurses were distressed, upset. They were sort of crying,” he said. “I think they must have known things were going downhill. We sort of had a hint then.”

Australian authorities say Patel left the country after the allegations against him were aired in March, in a letter from a senior nurse at the hospital, Toni Hoffman, that was tabled in the Queensland Parliament. His whereabouts are unknown.

He has a home in Portland, Oregon, but has not returned phone calls there from the AP.

Some reports have suggested he has returned to India. But Patel’s mother Mridulaben Patel, 89, said she hasn’t heard from him in some time.

“I understand that he is a very famous doctor in America and Australia, but about the charges against him that you are telling me now, I am in the dark,” she said, sitting in the bedroom of the family’s palatial home in the western province of Gujarat.

Patel, 55, comes from a wealthy family and attend the state-run M.P. Shah Medical College at Saurashtra University in Jamnagar city, earning a medical degree in 1973 and a master’s in surgery in 1976, university records show.

“In spite of the fact that he belonged to a powerful and rich family, Jayant was always modest and keen to work with the poor,” said Dr. R.U. Mehta, who oversaw some of Patel’s studies. “I cannot understand what exactly has gone wrong with him.”

Patel practiced in India before moving to the U.S, where he took up an internship and residency at the Rochester University School of Medicine in New York in 1979, a Medical Board of Queensland report says. He moved to Portland in 1989 and began working for Kaiser Permanente hospital.

In 1998, Kaiser banned Patel from conducting certain operations, including liver and pancreatic surgeries, and required him to seek a second opinion in complicated cases.

Kaiser spokesman Jim Gersbach said the hospital confirmed Patel had an unrestricted license before hiring him, and that he was well recommended by previous employers.

The Oregon Board of Medical Examiners made Patel’s restriction statewide in 2000, prompting New York authorities to ask Patel to voluntarily surrender his license for that state, documents from New York show.

The documents cited “gross … negligence on more than one occasion.”

Patel surrendered his New York license in 2001.

Despite the U.S. bans, Patel twice answered “no” when asked in applications to practice in Australia if he had ever been the subject of disciplinary action in another state or country, the Medical Board of Queensland said.

He gained an Australian license in 2003.

Co-workers at Bundaberg soon noticed disturbing signs.

Hoffman, the nurse who worked with Patel, told the AP he regularly failed to wash his hands between patients, was belligerent toward nursing staff and often resisted transferring patients.

In her letter tabled in parliament, she wrote: “Every time I see him walk into the unit … I feel sick because I just think ‘who’s he going to kill now, what’s he going to do now?'”

Mark Bramich believes bravado on Patel’s part contributed to his father’s death.

“I think he just kept having to do it himself, fix it up,” Mark said. “He was way out of his depth.”

—_

Associated Press reporters Rupak Sanyal in India and William McCall in Oregon contributed to this report.

Alcohol Merchandise Encourages Underage Drinking

Middle school kids who had promotional materials started drinking earlier

HealthDay News — Adolescents who collect and brandish promotional hats, shirts, bags and other merchandise displaying popular alcohol logos are far more likely to start drinking while still underage, according to a new study.

Researchers from the Dartmouth-Hitchcock Medical Center in Lebanon, N.H., were to present the finding Tuesday at the annual meeting of the Pediatric Academic Societies in Washington, D.C.

In light of the apparent connection, the authors of the study recommended that the alcohol industry officially halt the practice of distributing and selling alcohol-related paraphernalia — much as the tobacco industry did with tobacco-related items in 1998.

“This study shows that promotional items are related to early onset drinking, and I think the responsible thing to do would be for these industries to quit distributing them,” said Dr. James D. Sargent, study co-author from Dartmouth’s department of pediatrics.

According to the study authors, the alcohol industry currently spends more than $1 billion a year on all aspects of marketing — a figure that includes expenditures for such youth-oriented promotional items as baseball caps, backpacks and t-shirts.

Such teen-targeted branding flies in the face of the 1984 federal National Minimum Drinking Age Act, which set the drinking age in the United States at 21 years — the highest in the world. The legislation mandated that all 50 states prohibit the selling of alcohol to minors under the age of 21. Public possession of alcohol by minors was similarly made illegal.

However, the law did not actually outlaw underage drinking — allowing those under 21 to legally consume alcohol in private settings or for either religious or medicinal purposes.

According to 2003 figures issued by the National Institute on Alcohol Abuse and Alcoholism, almost half of all adolescents have had at least one drink — and more than one-fifth have been drunk — by the time they enter the eighth grade.

Beginning in 1999, Sargent and his colleagues examined the drinking behavior of this age group by focusing on more than 2,400 middle school students in Vermont and New Hampshire.

The adolescents ranged in age from 9 to 15 years — attending grades five through eight. The research team established that none of the children had ever had a drink at the onset of the study.

During follow-up telephone interviews conducted one to two years later, Sargent and his team found that 14 percent of the students said they now owned at least one alcohol-related promotional item. Of the 32 students who mentioned the particular branding of their promotional possession, 29 had beer-related ones.

The researchers further found that 15 percent of the students said they now drank alcohol to some degree.

Those students who owned alcohol merchandise were significantly more likely to start drinking alcohol than those who did not. More than 24 percent of those who owned promotional items said they consumed alcohol, compared with the slightly more than 12 percent of non-owners who said they drank.

The researchers noted that ownership of alcohol promotional items was associated with being at the older range of the student group, having peers who drank, having tried smoking, “sensation-seeking,” and doing less well in school.

“I think the beverage industry needs to take this seriously,” said Sargent. “There’s a tremendous amount of research showing that branded merchandise that the tobacco industry distributed clearly contributed to the teen smoking problem. There’s just no doubt about it. Looking at the branded merchandise distributed by the alcohol industry is a relatively new topic, but it’s such a similar situation that I would be surprised if multiple studies won’t show that this is true in this case as well.”

Sargent said the findings were a wake-up call for parents as well as the alcohol industry.

“For parents, you really shouldn’t allow your kids to have these things,” he cautioned. “Firstly, it increases the chance they will take up drinking at an early age. And secondly, when they wear them, they are a walking billboard. And you don’t want your kid to advertise Budweiser beer to kids.”

“For the wine and liquor industry,” he added, “the point is that these kind of promotional things related to smoking were shown to lead to increased smoking among teens. And the tobacco industry gave up putting out the items.”

Sargent said the alcohol industry should pick up on big tobacco’s cue — noting that he expects a larger national study of teens he is currently conducting to further underline the urgency for such action.

David Jernigan, research director at the Center on Alcohol Marketing and Youth at Georgetown University in Washington, D.C., echoes the sentiment that the alcohol industry must take responsibility for its influence on underage drinking.

“The bottom line is that even the industry agrees that peer pressure is critical in a kid’s decision to drink,” he said. “So it creates a whole set of walking billboards among the peer group at risk — among the underage peers. And that’s not helpful to efforts to reduce underage drinking.”

Attempts by HealthDay to reach representatives of the beer industry for comment were unsuccessful.

More information

Dartmouth-Hitchcock Medical Center

Center on Alcohol Marketing and Youth

For more on underage drinking, check out the National Institute on Alcohol Abuse and Alcoholism (www.niaaa.nih.gov ).

Trampoline Injuries Soar

An estimated 75,000 children are hurt each year, report finds

HealthDay News — Emergency room visits due to trampoline injuries have almost doubled since the early 1990s, new research shows.

An estimated 75,000 children are injured each year, and 91 percent of those injuries occur on backyard trampolines, said one of the study’s authors, Dr. James Linakis, a pediatric emergency physician at Hasbro Children’s Hospital in Providence, R.I.

“This problem has not gone away,” said Linakis, who added, “The home environment and even the school environment are not the place for trampolines. While not a popular idea, trampolines are really only appropriate in very select, heavily supervised environments,” such as a gymnastics school.

The findings were presented Sunday at the Pediatric Academic Societies’ annual meeting in Washington, D.C.

In 1999, the American Academy of Pediatrics (AAP), citing an increase in trampoline-related injuries, issued a policy recommending that trampolines not be used at home or for physical education classes at school.

Linakis said that, after the policy came out, he expected to see a drop in the number of trampoline injuries. Instead, he said, “We were still seeing a large number of trampoline injuries in the ER.”

And, that discrepancy is what triggered this study. Linakis and his colleagues reviewed national data on trampoline injuries collected by the U.S. Consumer Product Safety Commission for 2001 and 2002, and compared it to data collected for a study that looked at trampoline injuries from 1990 through 1995.

During the early 1990s, about 42,000 children hurt themselves badly enough each year to require an emergency room visit. But, by 2001-2002, that number had jumped to almost 75,000 annually.

At the same time, the number of injuries requiring hospital admission also jumped dramatically, from 1,400 each year in the early 1990s to 2,218 annually by 2001-2002.

Linakis said he’s not sure why the injury rate is going up so dramatically, but speculated, “Either we’re not getting the message out or we are getting the message out and people aren’t paying attention.”

The average age of the children injured was 9, and 53 percent were male. The most common injuries were bruises, cuts, fractures and dislocations, he said.

Linakis said the researchers weren’t able to collect information on exactly how the injuries occurred. But allowing more than one child on a trampoline at a time is a significant source of injury, he said.

Dr. Karen Sheehan, medical director for Injury Prevention and Research at Children’s Memorial Hospital in Chicago, said, “We haven’t done all we can at getting out the message” about the dangers of trampolines.

“Supervision is key to avoiding injuries,” said Sheehan. “While the AAP strongly recommends getting trampolines out of the home, we probably can’t get rid of them all, but we can make them safer.”

She said if you have a trampoline at home, make sure you’re always right there supervising children when they’re on the trampoline. Another way to avoid injuries is to make sure that only one child jumps at a time.

Nets that enclose the trampoline may help, but both Sheehan and Linakis said they simply haven’t been studied to see if they make trampolines safer. Sheehan pointed out that many injuries, such as broken bones, occur even when children don’t fall off the trampoline, and having a net won’t help avoid those injuries.

The U.S. Consumer Product Safety Commission, which reports that six children have died using trampolines since 1990, also recommends:

  • No children under 6 use a trampoline.
  • Don’t have ladder access to your trampoline because small children can then climb up unsupervised.
  • Don’t let older children attempt somersaults — doing so increases the risk of a serious head or neck injury.
  • Make sure the trampoline is nowhere near tree branches or playground equipment.

More information

Hasbro Children’s Hospital

The U.S. Consumer Product Safety Commission offers more information on trampoline safety.

Get an Agent Orange Exam: Protect Your Health

Some 371,307, or 14%, of the 2.6 million Americans who served on the ground in Vietnam have received physical examinations under VA’s registry.

Any and all Vietnam veterans who have not had an Agent Orange exam should do so immediately, according to VFW. Especially if they have cancer.

“If a Vietnam veteran has recorded documentation of a cancer that VA considers to be caused by Agent Orange exposure-such as prostate cancer-he will more than likely have a disability rating within two weeks,” says John McNeill, deputy director of VFWs National Veterans Service. “VA is very quick on that.”

Before visiting VA for an exam, McNeill says veterans should see a service officer for guidance. The service officer can explain the 12 conditions (see information box) that VA presumes to have been caused by Agent Orange exposure and that make a vet eligible for VA compensation.

Of the 12 diseases, McNeill says, VA grants most compensation claims for diabetes and prostate cancer. The two diseases are prevalent in the general population as well. Some 18.2 million people in the United States, or 6.3% of the population, have diabetes. And after skin cancer, prostate cancer is the most common form of cancer in American men.

VFW urges all Vietnam vets to take the exam, which is part of VA’s Agent Orange Registry program. By becoming part of the registry, Vietnam vets receive updates from VA on new diseases that have been added to the list or those that are being researched.

As of February 2005, some 371,307 Vietnam veterans had registered with VA’s program and taken an initial Agent Orange exam.

As required by law, VA reviews research on herbicide exposure every two years and adds any diseases that may have been caused by Agent Orange. The latest disease added was chronic lymphatic leukemia in January 2003.

Currently, VFW is pushing for melanoma-a deadly skin cancer-to be included. VFW delegates called for this last year when they approved Res. 698 at the organization’s national convention.

McNeill says it’s important for Vietnam vets who have diabetes or prostate cancer to get registered immediately because they could be losing VA compensation.

“VA gives veterans with an active cancer an initial 100% service- connected disability rating,” he says. “This rating stays in place while the vet undergoes treatment, then VA notifies the vet that he has 120 days to take a second exam. The whole process could take up to two years, during which time the vet could be collecting compensation.”

McNeill adds that if a veteran’s cancer is diagnosed as terminal, service officers often can obtain a VA disability rating within one day.

Ironically, McNeill-himself a Vietnam veteran-has not taken a VA Agent Orange exam.

“That’s because I know I don’t have any of the conditions that VA presumes to have been caused by Agent Orange exposure,” he says. “But if I was not a service officer, I wouldn’t know if I was eligible for compensation or not. That’s why it’s so important for Vietnam vets to consult a service officer. They can protect themselves-and their childrennow and into the future.” O

Contact Information

VFW encourages its members to consult a VFW service officer before taking a VA Agent Orange exam. For concerns or questions about VA health care, contact VFWs Tactical Assessment Center at

1-800-VFW-1899. VA operates a toll-free helpline for veterans with Agent Orange-related concerns at 1-800-749-8387. VA’s national toll-free number for information about VA benefits is 10 -8000 -827- 1000. Veterans can apply online for benefits at http://vabenefits. vba. va.gov/vonapp.

For more information about the Agent Orange Registry program, contact the registry physician at the nearest VA medical facility. VA facilities are listed online at www.va.gov, under “Facility Locator.”

VA also publishes the newsletter Agent Orange Review for Vietnam vets, their families and others with questions or interests about the herbicide. It is available online at www.va.gov/AgentOrange under “Agent Orange Review.” To get on the mailing list, send your name and address to:

Agent Orange Review

Austin Automation Center (200/397A)

1675 Woodward Street

Austin, TX 78772-0001

All Vietnam Vets Eligible for Exams

Since 1978, VA has offered free physical exams for Vietnam veterans based on their possible exposure to Agent Orange. Here is what VA provides:

* a pre-exam interview to determine where and when a veteran served in country;

* compilation of the veteran’s medical history;

* a physical exam;

* a series of basic laboratory tests, such as chest X-rays (if appropriate), urinalysis and blood tests;

* consultations with other health specialists, if needed;

* a post-exam interview to discuss results;

* a letter explaining the findings;

* a follow-up exam or additional lab tests, if needed; and

* notation of exam and test results in the veteran’s permanent medical file and Agent Orange Registry.

12 Diseases Linked to Agent Orange Exposure

VA offers compensation to Vietnam veterans suffering from any of the following conditions, as well as their children stricken with spina bifida.

Chronic lymphatic leukemia: An uncontrolled growth of white blood cells in the blood, bone marrow and lymphatic tissues.

Chloracne: A skin condition that appears similar to common acne found among teenagers.

Diabetes: Disorders in which the body has trouble regulating its blood sugar levels.

Hodgkin’s disease: Cancerous growth of cells in the lymph system.

Multiple myeloma: Cancer of white blood cells in bone marrow.

IMon-Hodgkin’s lymphoma: Cancers of the lymph nodes, spleen and other immune system organs.

Peripheral neuropathy: Damage to the nerves involved with sensation and feeling.

Porphyria cutanea tarda: A defective enzyme in the liver involved in producing the red pigment in blood cells.

Prostate cancer: Cancerous tumors on the male glands that surround the urethra at the bladder.

Respiratory Cancers: Cancerous tumors of the lung, larynx, trachea and bronchus.

Soft tissue sarcoma: Cancerous tumors in fat, muscles, nerves, tendons and blood and lymph vessels.

Spina bifida: Literally means “split or open spine” and is a birth defect that may affect children of Vietnam veterans.

Copyright Veterans of Foreign Wars of the United States May 2005

Outcome Studies in Type 2 Diabetes

Key words: Cardiovascular – Diabetes – Outcomes – Pioglitazone – PROactive – Thiazolidinediones

ABSTRACT

Background: Outcome studies are used to measure clinically meaningful primary end points, such as mortality and cardiovascular morbidity. However, few outcome trials have been conducted exclusively in people with diabetes; the majority of conventional diabetes trials use surrogate end points that may or may not translate into clinical benefits. Our current knowledge of the effects of pharmacotherapies on cardiovascular risk in patients with diabetes has been gained from subgroups included in large-scale studies. Several trials with lipid-modifying, antiplatelet and/or antihypertensive therapy, for example the recent Collaborative AtoRvastatin Diabetes Study, have included sufficient numbers of patients with diabetes to indicate that effective management can reduce cardiovascular risk in this patient population. The United Kingdom Diabetes Study and the Diabetes Control and Complications Trial provide important, but inconclusive data on the impact of glucose-lowering therapy on the incidence of cardiovascular complications in diabetes. Thiazolidinediones have only become available during the past few years, thus their effects were not assessed in these landmark trials. Ongoing studies in diabetic populations at high risk for further macrovascular events, such as the PROspective pioglitAzone Clinical Trial In macroVascular Events, have been designed to assess the effect of thiazolidinediones on cardiovascular outcome in patients with diabetes and should help to reinforce the importance of broad-based treatment of the multiple metabolic risk factors for cardiovascular disease in people with diabetes.

Scope: This paper (based upon MEDLINE and EMBASE literature searches in the year range 1990-2005) reviews what we have learned from outcome studies up to the end of 2004 and looks at what we hope to learn from ongoing studies.

Introduction

Those with metabolic syndrome ideally require an integrated approach to manage weight loss, glucose levels, hypertension and dyslipidaemia. The most effective and frequently used management options for the different underlying causes of the metabolic syndrome include:

* Preventing and managing obesity.

* Modulating lipid levels, e.g. LDL-cholesterol and triglyceride reduction as well as increase in HDL-cholesterol.

* Managing hypertension.

* Addressing subclinical inflammation.

* Treating insulin resistance.

Outcome studies are used to show if these management options deliver clinically meaningful benefits by using primary endpoints that are of direct importance to patients, e.g. overall mortality. However, there are few outcome studies that have tested the effects of pharmacotherapies on outcomes in diabetes and most data on endpoints related to macrovascular disease (e.g. all-cause death, cardiac death and cardiovascular morbidity) are from trials with surrogate end points or are subgroup analyses of cardiovascular outcome trials.

Nevertheless, there are important data from a small number of landmark outcome trials using lipid-lowering agents, antihypertensives and/or antiplatelets, but the United Kingdom Prospective Diabetes Study (UKPDS) is the only outcome study to date to assess specifically the effects of oral glucose-lowering agents on vascular risk reduction in patients with type 2 diabetes. Although we learned a considerable amount from this study, several questions were left unanswered.

In 2005, the results of PROspective pioglitAzone Clinical Trial In macro Vascular Events (PROactive), the first large-scale outcome study of an oral glucose-lowering agent in the management of type 2 diabetes, will be revealed.

This paper reviews what we have learned from outcome studies up to the end of 2004 and looks at what we hope to learn from ongoing studies. It is based upon MEDLINE and EMBASE literature searches using the search terms outcome trials, cardiovascular risk reduction, mortality, morbidity, statins, fibrates, diabetes, antihypertensives, antiplatelets and glucose-lowering and the year range 1990-2005.

Landmark outcome trials

Studies in people with diabetes

Several landmark trials provide important data on the impact of glucose-lowering therapy on the incidence of cardiovascular (CV) complications. There are also two completed studies that assess the impact of lipid-lowering therapy on reduction of surrogate macrovascular endpoints in a population of people with diabetes exclusively (Table 1).

The University Group Diabetes Program (UGDP) was one of the earliest outcome studies that was conducted in the 1960’s in patients with type 2 diabetes (n = 823) during an 8-year period. Five groups of patients were studied: diet plus placebo, standard insulin doses, variable insulin doses, tolbutamide and phenformin. The findings for the two insulin groups were inconclusive in terms of delaying or preventing CV complications and there were no significant differences in incidence of overall mortality in all of the groups1.

Table 1. Diabetes trials investigating cardiovascular outcome

The UKPDS is the most important clinical trial in patients with type 2 diabetes. During a 20-year period over 5000 patients with newly diagnosed type 2 diabetes were studied to determine whether intensive pharmacological blood glucose control could reduce the risk of diabetic micro- and macrovascular complications. A large number of endpoints, including myocardial infarction, heart failure, angina, renal failure and stroke, were measured. The study demonstrated that intensive glycaemic control of type 2 diabetes was effective in reducing HbA^sub 1c^ (which is a risk factor for vascular disease itself) and in all microvascular complications (25% decrease; median follow-up of 11.1 years). For every 1% decrease in HbA^sub 1c^ there was an associated 37% reduction in microvascular complications2,3. The reduction in the risk of macrovascular disease was less pronounced than the improvements in microvascular disease, with a modest 16% reduction in myocardial infarction that was of borderline significance3. However, in the metformin subgroup analysis (n = 342) there were significant reductions in diabetes- related deaths (42% reduction), any diabetes-related endpoint (32% reduction) and myocardial infarction (39% risk reduction)4.

In the Diabetes Intervention Study (DIS), 1139 people with type 2 diabetes were followed for 11 years to determine the effect of intensified versus conventional health education in improving metabolic control and reducing the level of coronary risk factors and incidence of ischaemic heart disease5. This study showed that triglycerides were an important risk factor for myocardial infarction. Clofibrate efficacy was assessed at 5 years within the intensified group (379 out of 761 patients) and decreased triglyceride levels6. Although clofibrate was effective in reducing triglycerides, it did not appear to prevent CV complications and myocardial infarction and mortality increased more with postprandial than with fasting hyperglycaemia. The incidence rate per 1000 for myocardial infarction was 30.3 for control subjects, 53.6 for the intensive group and 55.6 for the intensive group receiving clofibrate therapy.

The Diabetes Control and Complications Trial (DCCT) was a 10- year, multicentre, prospective randomized trial in 1441 people with type 1 diabetes [n = 726 in the primary prevention cohort and 715 in the secondary intervention cohort) designed to determine the effect of intensive management versus conventional management on the prevention of retinopathy (the primary outcome) and other vascular complications in type 1 diabetes7. The study found that intensive glycaemic control reduced HbA^sub 1c^ by 1.9% over the 10-year period, with a 76% decrease in the risk of retinopathy in the primary prevention cohort and a delayed progression in 54% of those with established retinopathy. The risk of albuminuria and microalbuminuria were also reduced by intensive insulin therapy (decreases of 54% and 39%, respectively). There were also non- significant risk reductions for all macrovascular CV events combined (41%).

The Diabetes Atherosclerosis Intervention Study (DAIS) is the first clinical trial involving randomized lipid-lowering therapy in a population of people with diabetes exclusively. In this 3-year study in 418 patients with diabetes and characteristic diabetic dyslipidaemia, with or without previous cardiac interventions, fenofibrate reduced coronary atherosclerosis (measured by angiography) by 40%. However, the study was not powered to detect significant differences in clinical cardiac endpoints8.

The Collaborative AtoRvastatin Diabetes Study (CARDS) is the first ever primary prevention study of cholesterol-lowering specifically in patients with type 2 diabetes at low risk for CV events (they had no previous history of CVD and had normal LDL- cholesterol levels). Nearly 3000 patients with type 2 diabetes were randomized to either atorvastatin or placebo. The trial was halted 2 years early (mean duration of follow-up of 3.9 years) as the prespecified efficacy endpoint criteria (a significant difference in favour of atorvastatin) was met during an interim analysis. Those patients treated with the statin had a 37% reduction in the risk of a major CV event. In addition, acute coronary event rate was reduced by 36% and stroke by 48% in the atorvastatin-treated group9.

\In addition, two recent trials, the Irbesartan Diabetic Nephropathy Trial (IDNT) and the Reduction of Endpoints in NIDDM with the Angiotensin II Antagonist Lorsartan (RENAAL), showed the effects of antihypertensives on microcirculatory endpoints in patients with type 2 0 diabetes10,11. IDNT looked at the effects of therapy with an angiotensin II receptor antagonist (irbesartan); a calcium channel blocker (amlodipine) or placebo as add-on to standard antihypertensive therapy in patients with type 2 diabetes (n = 1715). The composite microvascular endpoint of doubling of baseline serum creatinine concentration, the onset of end-stage renal disease or death from any cause (surrogates of progression of diabetic nephropathy and development of end-stage renal failure) were improved by the use of the angiotensin II antagonist. However, there were no significant differences between groups for the secondary CV composite endpoint (death from CV causes, non-fatal myocardial infarction, heart failure, neurological defects caused by a cerebrovascular event or lower limb amputation)10.

Subgroup analyses of patients with diabetes in large outcome trials

Several trials with lipid-modifying, antiplatelet and/ or antihypertensive therapy have included sufficient numbers of patients with diabetes to indicate that effective management of dyslipidaemia, endothelial dysfunction and/or hypertension can slow the progression of coronary atherosclerosis and reduce the risk of future coronary events in patients with diabetes (Table 2).

Outcome trials using statins

Data from secondary prevention studies confirm a link between cholesterol levels and coronary heart disease (CHD) risk and the benefits of statins on CV risk factor modification apply to subgroups with diabetes.

The Heart Protection Study (HPS) was a randomized, placebo- controlled trial in over 20 000 people with CHD, other occlusive arterial disease or diabetes (mean follow-up of 5 years). The study aimed to determine the potential long-term benefits of cholesterol- lowering therapy (simvastatin) in people at increased risk of CHD. Approximately 6000 people with diabetes (mainly type 2) were enrolled and one-third of these also had concomitant CHD. In the subgroup with diabetes, simvastatin reduced the risk of myocardial infarction, stroke and other vascular complications by one-third12.

Four smaller studies (Long-term Intervention with Pravastatin in Ischaemic Disease [LIPID]13, Cholesterol And Recurrent Events [CARE]14, Scandinavian Simvastatin Survival Study [4S]15,16 and the West of Scotland Coronary Prevention Study [WOSCOPS]17), which were conducted in patients with CHD, also assessed data from subgroups of patients with diabetes.

The LIPID study was conduced in patients with a history of myocardial infarction or unstable angina and a wide range of plasma cholesterol levels on entry, with a mean follow-up of 6.1 years. When treated with pravastatin, the subgroup of patients with diabetes (n = 782 out of the total study population of 9014 people) showed a 19% reduction in CHD risk of death and non-fatal myocardial infarction (the primary outcome) compared with a decrease of 25% in non-diabetics13.

Table 2. Landmark trials with subgroup analyses of patients with diabetes

The CARE study looked at pravastatin treatment in patients with prior CHD and average LDL-cholesterol levels. Analysis of the subgroup of patients with a history of diabetes (n = 586 out of 4159) showed a 25% reduction in relative risk of coronary events after 5 years of treatment with pravastatin14.

In 4S (median follow-up of 5.4 years), simvastatin therapy resulted in a risk reduction of 43% in total mortality (the primary endpoint) and 55% risk reduction in major CHD events in a subgroup of 202 patients with diabetes15. This did not reach statistical significance, probably due to the small patient number. A post-hoc analysis, extending this into a larger cohort of 483 patients that met ADA criteria for diabetes, showed a 42% reduction in major CHD risk and a 21% reduction in total mortality with simvastatin16. It is thought that the greater effect of statin therapy on CHD risk in 4S than in the CARE and LIPID studies may be due to more marked effects in those with elevated LDL-cholesterol concentrations.

WOSCOPS examined the development of a new diagnosis of diabetes in men with moderate hypercholesterolaemia, but no history of myocardial infarction. The primary endpoint was non-fatal myocardial infarction or death from CHD. Only 1% of the study population had diabetes on entry into the study so no subgroup information is available; however, a retrospective subgroup analysis showed that the risk of developing diabetes was reduced by about 30% in patients receiving pravastatin17. One hundred and thirty nine of the 6595 men developed diabetes during the 3.5-6.1 year follow-up.

Outcome trials using fibrates

There are two analyses of fibrate therapy (gemfibrozil) in people with diabetes from larger studies in CHD. In the first, the Veterans Affairs High-density lipoprotein Intervention Trial (VA-HIT), the role of fibrate therapy in preventing CHD events in men with known CHD was studied (median follow-up of 5.1 years). A total of 769 people out of the 2531 with CHD also had diabetes. Gemfibrozil use in this subgroup of patients with diabetes reduced the composite endpoint of CHD death, non-fatal myocardial infarction or confirmed stroke by 32% and stroke by 40%18.

Of the 4081 males in the Helsinki Heart Study, 135 patients had diabetes. Gemfibrozil reduced CV risk by 68% in this subgroup, however, there was no statistically significant difference between the gemfibrozil and placebo groups, with a low incidence of cardiac events in both groups (3.4% in the gemfibrozil group and 10.5% in the placebo group), probably due to the small patient number19.

Outcome trials using antihypertensives

Studies assessing the effects of antihypertensive agents on CHD risk have also shown reductions in risk rate in people with diabetes. In particular, the Heart Outcomes Prevention Evaluation (HOPE) trial, GISSI-3 and the UKPDS, all showed that blood pressure lowering interventions were beneficial in reducing CHD and stroke in patients with diabetes20-22.

The HOPE study assessed the effects of the ACE inhibitor, ramipril, in more than 9500 patients with a high risk for CV events. In the population of patients with diabetes (n = 3577; The MICRO- HOPE Study), ramipril provided significant clinical benefits on the primary outcome (25% risk reduction of combined myocardial infarction, stroke or CV death) and also on other outcomes (reductions of 22% in myocardial infarction, 33% in stroke, 24% in total mortality and 37% in CV death)20. The effect appeared to be independent of, and additive to, the effects of antiplatelet and lipid-lowering agents.

In GISSI-3, 2790 of the 18131 patients enrolled had diabetes. The ACE inhibitor, lisinopril, was given within 24 hours from the onset of symptoms of acute myocardial infarction and continued for 6 weeks to see if mortality and morbidity could be reduced. Treatment with lisinopril reduced 6-week mortality (30% risk reduction)21.

In the UKPDS, intensive blood pressure control with either an ACE inhibitor or a β-blocker, gave a 37% improvement in microvascular endpoints with a 21% reduction in myocardial infarction and 44% in stroke in a subgroup of people with hypertension22. Although this subgroup was very small, it does suggest that there are benefits of combining treatments for the modification of multiple CV risk factors in patients with diabetes.

Outcome trials using antiplatelets

Only one outcome study using antiplatelet therapy has assessed effects in a subgroup of patients with diabetes. Hypertension Optimal Treatment (HOT) is the largest study of the effects of aspirin on major CV events (n = 18 790). Mean follow-up was 3.8 years. All patients received the long-acting calcium antagonist, felodipine, and ACE inhibitors, beta-blockers and/ or diuretics were added according to a preassigned regimen of intensive versus conventional hypertension management. The effects of add-on therapy with aspirin were compared with placebo. In patients with diabetes, those intensively treated experienced a 51% decrease in major CV events. Aspirin reduced major CV events by 15% and myocardial infarction by 36% in the total population23.

Outcome trials using combination therapies

Controlling one risk factor alone does not eliminate the risk of CHD in patients with type 2 diabetes and the combined use of two or more agents with complementary mechanisms of action may provide greater improvements in CV risk factors than the use of single agents.

The Multiple Risk Factor Intervention Trial (MRFIT) provided evidence that tight control of hypertension, cholesterol and smoking reduced cardiovascular mortality in patients with type 2 diabetes24. In addition, the small, 9-year study (mean follow-up of 7.8 years) Steno-2, looked at reduction in risk factors for CVD in 160 patients with type 2 diabetes and microalbuminuria given intensive multifactorial, stepwise treatment with an ACE inhibitor or an angiotensin II receptor blocker (regardless of blood pressure levels), a lipid-modifier (statin or fibrate) and a glucose- lowering agent, in addition to aspirin, vitamin supplements plus diet and lifestyle changes25. Intensively treated patients were much more likely to achieve therapeutic goals and had a mean reduction in macrovascular disease of 53% relative to conventional therapy given in accordance with national guidelines. This large reduction in CV disease risk is substantially higher than that observed in previous single intervention trials using lipid-modulators or antihypertensives, suggesting that targeting multiple risk factors is necessary in people with diabetes.

New outcome trials in people with diabetes

A number of outcome studies of macrovascular endpoin\ts in patients with type 2 diabetes exclusively are currently underway.

Glucose-lowering studies in patients with diabetes

Thiazolidinediones (TZDs) have only become available during the past few years, thus their effects have not been reported previously from any long-term outcome-based trials. A series of studies evaluating the effects of pioglitazone on the progression of atherosclerosis are ongoing.

The PROspective pioglitAzone Clinical Trial In macro Vascular Events (PROactive) in patients with type 2 diabetes and clinical evidence of macrovascular disease is one of these studies26.

Rationale for PROactive

Pioglitazone is known to improve glycaemic control and also possesses additional properties that may have an impact on clinical vascular outcomes. Assessment of the non-hypoglycaemic effects of pioglitazone show improvements in several CV risk factors, including modification of lipids and independent predictors of CHD (e.g. microalbuminuria and plasminogen activator inhibitor1) and reductions in carotid intima-media thickness that could decrease morbidity for macrovascular disease27-34. PROactive tests the hypothesis that pioglitazone reduces total mortality and macrovascular morbidity in high-risk patients with type 2 diabetes and also examines its effects on individual risk factors for vascular complications.

Design of PROactive

PROactive is a randomized, double-blind, placebo-controlled outcome study in more than 5000 patients with type 2 diabetes, managed with diet and/or oral blood glucose-lowering drugs, who are at increased CV risk. Patients are randomized to receive ‘add-on’ therapy with pioglitazone (increased stepwise from 15 mg to 30 mg to 45 mg, dependent on tolerability), or placebo, to their existing medications for management of hyperglycaemia, dyslipidaemia, thrombosis and hypertension, which are to be continuously optimized throughout the trial according to the International Diabetes Federation (IDF) Europe Guidelines35 to allow patients to receive the best possible available therapy. The primary endpoint is the time from randomization to occurrence of a new macrovascular event or death. Follow-up is estimated to span 4 years and the first presentation of the results is expected in late 2005.

Summary of the baseline PROactive data

A total of 5238 patients have been randomized from 19 European countries. At entry into the study, patients were a mean age of 61.8 years with type 2 diabetes for a mean of 9.5 years. Patients met one or more of the entry criteria of: a history of myocardial infarction (46.7%), coronary artery revascularization (30.8%), or stroke (18.8%) for ≥6 months, acute coronary syndrome for ≥ 3 months (13.7%), other evidence of coronary artery disease (48.1%) or symptomatic peripheral arterial disease (19.9%). Half (48.5%) of the patients had two or more of these risk factors. In addition, three- quarters (75.4%) had hypertension. Almost all patients were on CV medications (95%) and 84% were on antiplatelet therapy. However, only half of the patients (52%) were receiving lipid-lowering therapy (mainly statins) and 63% were receiving ACE inhibitors. With respect to glucose-lowering therapies, 60.9% and 61.5% were taking metformin or a sulfonylurea, respectively, and 33.6% were using insulin in addition to oral glucose-lowering drugs.

Potential implications of PROactive

The cohort of patients enrolled in PROactive is a typical type 2 diabetic population at high risk for further macrovascular events. The characteristics of this population are ideal for assessing the ability of pioglitazone to reduce the cardiovascular risk of patients with type 2 diabetes. Despite the fact that the patients entered into the PROactive study are at high risk for CV events, it is clear from the baseline data that not all patients were receiving adequate therapies during everyday clinical practice. Hopefully, the results of the PROactive study will go someway to helping clinicians understand the unique nature of vascular disease in diabetes and the importance of broad-based treatment of the multiple metabolic risk factors for CVD in people with diabetes.

RECORD

The Rosiglitazone Evaluated for Cardiac Outcomes and Regulation of Glycaemia in Diabetes (RECORD) study is assessing whether the combination of rosiglitazone plus either metformin or sulfonylurea may be superior to the combination of metformin plus sulfonylurea for cardiovascular outcomes in people with type 2 diabetes. This 6- year study, planned to be completed in 2009, involves 3966 subjects with diabetes inadequately controlled on metformin or sulfonylurea36.

Statin studies in patients with diabetes

A primary prevention strategy is currently being tested in the Atorvastatin Study in Preventing Endpoints in type 2 diabetes (ASPEN).

Fibrate studies in patients with diabetes

The Fenofibrate Intervention and Event Lowering in Diabetes (FIELD) study involves more than 9000 people with type 2 diabetes. Patients have been randomized to fenofibrate or placebo. The primary endpoint of this study is coronary mortality and completion is due in early 200537.

Conclusions

There are few data on the effects of pharmacological intervention on the risk of CV events in patients with diabetes exclusively. Most of what we know to date comes from subgroups of people with diabetes that have been included in large-scale, landmark trials. There is a growing body of evidence that demonstrates the impact of lipid- lowering agents on CV outcome in subgroups of people with diabetes. A recent study, CARDS, has assessed the use of a statin in the primary prevention of CV events exclusively in patients with diabetes. Randomized clinical trials suggest that the TZDs have a positive effect on diabetic dyslipidaemia, but no studies have so far investigated if this will translate into clinical outcome benefits. Ongoing trials, such as PROactive, have been designed to look at the influence of glycaemic control on CV outcome in patients with diabetes and data are expected in late 2005.

Acknowledgements

I have received consulting fees/honoraria for lectures/ consultation from Bayer-AG, GlaxoSmithKline, Astra-Zeneca, Merck, Sharp and Dohme, Takeda, Aventis, Sanofi and local healthcare organizations in Germany.

I would like to thank Takeda for supporting this supplement with an educational grant. The views presented in this article are entirely my own and have not been influenced in any way by Takeda, nor has Takeda been involved in its preparation.

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31. Matthews DR, Charbonnel BH, Hanefeld M, Brunetti P, Schernthaner G. Long-term therapy with addition of pioglitazone to metformin compared with the addition of gliclazide to metformin in patients with type 2 diabetes: a randomized, comparative study. Diabetes Metabol Res Rev 2005;In press [EPub: 15 Jun 2004]

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37. The FIELD Study Investigators. The need for a large-scale trial of fibrate therapy in diabetes: the rationale and design of the Fenofibrate Intervention and Event Lowering in Diabetes (FIELD) study. Cardiovasc Diabetol 2004;3:9 [Accessed online at www.cardiab.com/content/3/1/9 on 24 January 2005]

CrossRef links are available in the online published version of this paper: http://www.cmrojournal.com

Paper CMRO-2863C_4, Accepted for publication: 04 February 2005

Published Online: 07 March 2005

doi:10.1185/030079905X36477

M. Hanefeld

Centre for Clinical Studies, GWT Technical University, Dresden, Germany

Address for correspondence: Direktor Prof. Dr. med. Habit. M. Hanefeld, Forschungshereich Endokrinologie und Stoffwechsel, Zentrum fur Klinische Studien, GWT-TUD mbH, Fiedlerstrasse 34, U1307 Dresden, Germany. Tl.: +49-3514-40-06-82; Fax: +49-3514-40-06-81; email: [email protected]

Copyright Librapharm 2005

Prostate Cancer and the Principles of Hormone Therapy Treatment

Abstract

This article discusses the use of hormone therapy for the treatment of prostate cancer, the most common male cancer. Key to managing this disease is understanding the processes responsible for maintaining prostate growth and hormonal homeostasis in male development. The individual mechanisms of action are outlined and demonstrated for each type of treatment, alongside the effects of these treatments. Each therapeutic option is discussed in conjunction with current key research findings and implications for practice are outlined.

Key words: * Male reproductive system and disorders * Men’s health * Prostate cancer

Prostate cancer is the most common male cancer. It accounts for over 27 000 cases in the UK a year. It is the second most common cause of death from cancer in males after lung cancer, with approximately 10 000 per year. The lifetime risk for being diagnosed is one in 14 and the incidence is rising annually (Cancer Research UK, 2004).

Despite its prevalence, the natural history of the disease is still relatively unknown, with many aspects of its clinical progression being poorly understood (Bono, 2004). However, since the 1940s and the Nobel prizewinning work of Dr Charles Huggins, it is known that prostate cancer responds to manipulation of the male hormone testosterone, either by surgery or pharmacological intervention. Hormonal manipulation has become the prime therapeutic strategy for patients in whom the prostate cancer is not considered curable with local radical therapy (Anderson, 2003).

This article will outline the pathogenesis of the disease, ways in which hormone therapy can be used and issues relating to the impact on the individual’s quality of life. Such treatments will be discussed alongside the role now commonly undertaken by many nurses in its delivery and patient surveillance.

Functions of the prostate gland

The prostate is about the size of a chestnut and lies at the base of the bladder, wrapped around the urethra. Although the prostate gland is known to be involved in stopping retrograde ejaculation, its exact role remains unclear (Emberton and Mundy, 1999). The prostate acts as a secondary sex organ, and its regulation and development are under the control of hormones (androgens).

Similar to breast tissue in females, the prostate gland consists of predominantly three types of cells: basal, epithelial or secretory and smooth muscle cells. Basal cells separate secretory cells from the basement membrane (Newling, 1997). The epithelial or secretory cells of the prostate are responsible for the production of a wide variety of substances, mainly enzymes and nutrients, which help in the mobility, feeding and penetration of the sperm into the female egg. The prostate also produces small concentrations of prostaglandins which may play a role in the stimulation of cellular activities of the gland itself and have an influence on the erectile tissue (Pryor, 1999). The stroma (the supporting fibromuscular connective tissue) of the prostate consists of smooth muscle cells and fibroblasts.

The prostate also has a profuse blood supply, and somatic as well as parasympathetic and sympathetic nerve fibres, an important aspect in prostate surgery when considering the preservation of erectile function in the patient. There are spaces around these nerve fibres called perineural spaces, where prostate cancer cells can migrate. With the migration of prostate cancer cells causing perineural invasion, the cells are able to wrap around nerves and travel through them in the same manner cars travel on a road, as the path of least resistance. Perineural invasion alone, however, does not represent extra prostatic extension, i.e. spread outside the prostate (Bostwick, 1999). It does, however, seem to represent an important predictor of early outcome in patients with organ- confined prostate cancer treated by prostatectomy (Endrizzi and Seay, 2000).

Prostate-specific antigen

One of the most widely known enzymes that is secreted from the cells of the prostate is prostate-specific antigen (PSA). PSA is a glycoprotein which is found in both benign and cancerous conditions of the prostate, and is responsible for the liquefying of seminal fluid to aid its mobility. It is produced almost entirely from the epithelial component of the prostate gland (Belledegrun et al, 1998). In order to measure it a blood sample is taken. Importantly, the PSA test is not a diagnostic test; while PSA is specific to the prostate, it is not specific to prostate cancer; those with an elevated PSA will require a transrectal ultrasoundguided prostate biopsy (TRUS) to obtain tissue on which a diagnosis may be made (NHS Cancer Screening Programmes, 2002). Its discovery in the early 1980s has revolutionized the entire approach to the management of prostate cancer (Brawer and Chetner, 1992).

With the disruption of the prostate gland’s cellular structures and barriers by different dis ease processes, especially prostate cancer, PSA gains access to the systemic bloodstream (Kirby, 2002). While there is no debate that early detection and aggressive treatment is the only way to cure the patient with significant disease, the debate about PSA screening is ongoing, primarily because of the risk of over detection and treatment of clinically insignificant disease (Potter and Emberton, 2003). It must be remembered that despite one-third of men over 50 years and a half of 80 year olds having a small focus of cancer in their prostate glands, only 4% of men will die from the disease (NHS Cancer Screening Programmes, 2002).

Apart from prostate cancer, the PSA levels can also be elevated in other conditions. Prostatitis and urinary-tract infections, trauma to the prostate seen at the time of catheterization, prostatic biopsies, recent ejaculation (within 48 hours) and benign prostatic hyperplasia (BPH) all lead to a rise in the PSA level (Brawer and Kirby, 1999). PSA as a tool to identify prostate cancer is therefore limited by these above confounding factors.

PSA is also limited by its sensitivity, the number of people who have the disease who have an abnormal test result, and specificity, the number of people without the disease who have a normal result (Potter and Emberton, 2003). Various strategies have focused on trying to enhance these parameters, to reduce the number of falsely positive test results, resulting in unnecessary further investigations, anxiety and financial cost (NHS Cancer Screening Programmes, 2002).

Table 1. Age-related reference ranges

Age-related reference ranges

The rationale for establishing age-specific PSA referenced ranges is based on a number of factors. First, PSA levels increase with age, which is more likely owing to the fact that there is greater leakage of PSA from the prostate epithelium with advancing age. second, the incidence of prostate cancer increases with age. Third, it is theorized that using a lower PSA cut-off point in younger men will allow increased sensitivity, while selecting a higher cut-off point in older men will enhance specificity (Brawer and Kirby, 1999).

The prostate cancer risk management programme recommends that the cut-off values in Table ? are used for the PSA test, although there is a single cut off of 4.0 ng/mL used in some areas (Department of Health, 2002). However, within men with a PSA of 4.0-10.0 ng/mL the so called ‘diagnostic gray zone’, the ability of PSA to distinguish prostate cancer from benign conditions, such as BPH and prostatitin’s, is only 18-25% (Catalona et al, 1993). Another emerging issue is the rising incidence of prostate cancer in the low PSA levels (less than 4.0 ng/mL). Studies have reported an incidence of between 24 and 26.3%, when the PSA levels were between 2.5 and 4.0 ng/mL (Djavan et al, 1999;Babaian et al, 2000).

Endocrinology of the prostate gland

The male sex hormone testosterone is the most important androgen in men, responsible for characteristics such as facial hair, sexual development in puberty, deepened voice and increased muscle bulk and bone mass (Marieb, 2003). Testosterone exerts its effects by binding to androgen receptors on target organs such as the prostate gland, and within the prostate it is converted to dihydrotestosterone (DHT) by the enzyme 5-alpha reductase. Testosterone and its more potent metabolite DHT are essential for normal prostate growth and are therefore thought to play a role in the development of prostate cancer (Kirby et al, 2001). Prostate cancer almost never develops in men castrated before puberty, or in individuals deficient in 5- alpha reductase, a syndrome called male pseudohermaphroditism (Brawley, 2003).

The normal function of the prostate gland and its cellular growth is maintained and regulated through a delicate balance by the hypothalamus, the pituitary gland, testes and adrenal glands (Kirby et al, 2001).

Hypothalamic-pituitary-testicular pathway

The hypothalamus caps the top of the brain-stem and is the main control centre of the body. It is vitally important for overall body homeostasis, with few tissues in the body escaping its influence (Marieb, 2003). Its main roles are shown in Table 2.

Table 2. Homeostatic functions of the hypothalamus

Stimulating sexual development and the production of testosterone begins with the hypothalamus and the release of a substance called luteinizing hormone-releasing hormone (LHRH). LHRH is secreted \in a pulsatile fashion by the hypothalamus for a few minutes at a time, once every 1-3 hours (Pryor, 1999). However, there is some disruption as one becomes older to the regularity and time span of the pulses (Keenan and Veldhuis, 2001). Although there is no dramatic reduction in sex steroid production in middle-aged men there is a small reduction in testosterone in the bloodstream, a proportionally larger increase in sex binding hormone globlulin in later life, and so levels of free, biologically active testosterone decline with age (Francis, 1999).

The increase in LHRH stimulates the anterior pituitary to secrete two key hormones: luteinizing hormone (LH) and follicle-stimulating hormone (FSH) responsible for stimulation of the Leydig cells of the testes to produce testosterone and sperm production. Testosterone is then converted into its more potent form DHT, by the enzyme 5-alpha reductase and male oestrogens, in the peripheral tissues and also in the prostate, thus promoting prostate cell growth (Griffiths et al, 1991).

Testosterone circulating throughout the body prevents the release of LHRH from the hypothalamus through negative feedback inhibition. This means that when levels of testosterone fall, LHRH is released in the hypothalamus leading to increased levels of LH and subsequently of testosterone. With a rise in testosterone levels, there is a decrease in the release of LHRH. This pathway is responsible for producing 95% of the testosterone produced daily.

Hypothalamic-pituitary-adrenal pathway

The adrenal glands are the secondary source of androgens. With the stimulation of the pituitary gland conies the release of corticotropin from the adrenal cortex, after stimulation by corticotropin-releasing hormone (CRH) which is secreted by the hypothalamus. Corticotropin is responsible for the regulation of adrenal androgens that are converted to testosterone and DHT and oestrogens in the peripheral tissues and the prostate, which again promote prostate cell growth. The adrenal glands indirectly contribute up to 5% of daily testosterone production.

Figure 1. Endocrine regulation of prostate growth. LHRH = luteinizing hormone-releasing hormone; CRH =corticotropin-releasing hormone; LH = luteinizing hormone; FSH = follicle-stimulating hormone; ACTH = adrenocorticotropic hormone; T-DHT = testosterone- dihydrotestosterone.

While testicular function and production of testosterone is intact, the adrenal androgens are not clinically important (Kirby et al, 1996). They are important, however, when usual testosterone production is inhibited in prostate cancer treatment. The elimination of the testicular androgens by surgical or medical treatment leaves an opportunity for the adrenal androgens instead to stimulate the growth of prostate cancer cells (Newling, 1997). Figure 1 refers to the endocrine regulation of prostate growth.

Hormonal therapy for prostate cancer: therapeutic options

For growth of prostate cancer cells to continue, stimulation is required by androgens. The primary strategy of hormonal therapy is therefore to decrease the production of testosterone by the testes or block the action that testosterone has on the growth of the cells from the adrenal glands and the testes. Hormonal therapy cannot cure prostate cancer, instead it aims to slow down the growth of the prostate cancer and reduce its overall size. Androgen stimulation of prostate cancer growth may be prevented in a number of different ways (Kirby, 2002).

Bilateral orchidectomy

Bilateral orchidectomy is the surgical removal of both of the testes, which are responsible for the production of 95% of the body’s testosterone. Since the testes are the major source of testosterone in the body, Garnick (1993) argues that this procedure is classified as hormonal therapy rather than surgical treatment. The goal of this procedure is to deprive the prostate cancer cells of testosterone, thereby causing the cancer to shrink and/or prevent further growth of the tumour by removing the source on which it depends. It reduces about 95% of circulating testosterone levels and a dramatic tumour response frequently occurs within 24 hours (Shroder et al, 2000). With this procedure the patient will become infertile, unable to produce sperm, and the loss in testosterone will cause erectile dysfunction, loss of libido and hot flushes. It can cause a reduction in bone pain if there is metastatic disease.

LHRH analogues

LHRH analogue injections mimic the action of LHRH and act by occupying a very high proportion of the LHRH receptors on the pituitary gland cell membrane. This initially leads to a rise in LH, resulting in a short-lived increase in testosterone production by the testes which can produce a flare-up of the disease and an increase in the patient’s existing symptoms (phase 1). However, as a result of constant occupation of the LHRH receptors on the pituitary cell by the continuing administration of this drug, a process known as down regulation occurs. This is a reduction in LHRH receptors, with accompanying densensitization, whereby there is a suppression of LHRH synthesis and excretion (Anderson, 2003).

Although new receptors are produced and appear on the cell surface of the pituitary gland, the continuing administration of the treatment causes them to become occupied immediately and to disappear. In this way, LHRH analogue treatment prevents the reappearance of LHRH receptors and constantly inhibits the production of LH and FSH from the pituitary gland and testosterone from the testes (phase 2). Testosterone suppression leads to shrinkage of the prostate tumour as shown in Figure 2. Tumour response is defined by testosterone suppression: usually 2-4 weeks.

With the new development of LHRH antagonists such as abarelix and cetrorelix as injection therapies for the treatment of prostate cancer, additional benefits are already being seen. As antagonists they occupy LHRH receptors, but do not stimulate them; there is therefore an absence of disease flare, rapid down regulation, and no need for combination therapy with an anti-androgen because of its binding, and blocking of the LHRH receptors in the pituitary gland (Weckerman and Harzmann, 2004). Table 3 shows LHRH analogue injections.

Anti-androgens

Anti-androgens are tablet-form treatments that block the effect of testosterone and its more potent form DHT binding to the androgen receptors in the prostate, in the presence of normal or increased levels of testosterone. They do not, however, prevent testosterone production (Griffiths et al, 1991). These orally administered drugs are classified by their chemical structure as either steroidal or non-steroidal (Table 4). Anti-androgens can be used in either combination therapy with LHRH analogue or as primary therapy.

Table 3. LHRH analogue injections

The steroidal anti-androgens have a dual mode of action, being both progestational and anti-androgenic in action (Figure 3). They block androgen receptors in the prostate and exert a negative feedback action on LH secretion, thus lowering the levels of testosterone production and the effect on the cancer cells in the prostate. Consequently, these medications are generally associated with loss of libido and erectile dysfunction (Shroder et al, 2000).

Non-steroidal anti-androgens or ‘pure anti-androgens’ block androgens from binding at the receptor sites of the prostate and also at the level of the hypothalamic-pituitary axis, where androgens normally elicit a negative feedback control on the release of LH (Figure 4). This leads to an increased LH output and a subsequent increase in the synthesis and secretion of testosterone. The increased conversion of testosterone results in an increased level of oestrogens, which have a direct effect on breast tissue, often causing enlargement of the breast, and increased sensitivity and sometimes pain around the nipples (Anderson, 2003). As they do not suppress testosterone synthesis, non-steroidal anti-androgens allow potential preservation of sexual interest and activity (Mason and Moffat, 2003).

Figure 2. Mechanism of action: luteinising hormone-releasing hormone (LHRH) analogues; LH = luteinizing hormone.

Oestrogens

Oestrogens are hormones found in men and women, although they are more widely known to control female sexual development, regulate the menstrual cycle and maintain pregnancy (Marieb, 2003). In prostate cancer they are principally derived from the peripheral conversion of testosterone in the tissues and from secretion by the testes in men with or without prostate cancer (Droller, 1997).

As treatment, they exert their effect by negative feedback, causing a reduction in the secretion of LHRH by the hypothalamus, thereby preventing the release of LH from the pituitary (Figure 5). As a result of non-secretion of LH, there is no action of the Leydig cells of the testes and hence no secretion of testosterone. There is also evidence to suggest that they have a direct cytotoxic effect (either killing the cancer cells or stopping them from multiplying) on the tumour (Kirby et al, 2001).

Indications for hormone therapy

Localized prostate cancer

Localized prostate cancer refers to prostate cancer that is confined within the capsule of the prostate with no evidence of spread. Such tumours are often too small to be palpable on rectal examination and may only be detected by transrectal ultrasound and needle biopsy of the prostate (Anderson, 1999). They may also be diagnosed following histological examination of the prostate ‘chips’ removed during a transurethral resection of the prostate (TURP) for bladder outflow obstruction presumed to be caused by benign prostatic enlargement.

Although the most appropriate management of patients with this disease is controversial, the aim is cure, whether eliminating the cancer or preventing death from it (Anderson, 2003).

Radical prostatectorny and radical radiotherapy

Radical prostatectomy is mostly un\dertaken as a curative procedure and involves the surgical removal of the entire prostate, the seminal vesicles and a variable amount of adjacent tissue. From the point of first-line treatment for localized disease, surgery and radiotherapy are the two principal treatment options alongside PSA surveillance.

Radical radiotherapy as a curative procedure involves a 6-week course of treatment. It offers a particular advantage in patients who are unsuitable for surgery because of co-morbidities, such as heart or lung problems, or evidence of extraprostatic extension of the cancer (Bono, 2004).

A short course (3 months) of hormonal drug therapy may be used before radical prostatectomy or radiation therapy to reduce the size of the prostate cancer (volume reduction). This is called neoadjuvant hormonal therapy.

While hormonal treatment before radical prostatectomy has been shown to reduce prostate cancer tissue volume and the incidence of positive areas of tumour following surgery, the results have not demonstrated any improvement in survival (Soloway et al, 2002).

With radical radiotherapy there is emerging evidence to suggest that the effect of radiotherapy as a curative treatment can be enhanced by 3 months pre-treatment with an LHRH analogue, with a clear improvement in overall survival (Bolla et al, 2002). This also helps to reduce the area to which radiotherapy is given, with a more defined area of treatment, reducing the effects such as diarrhoea and cystitis often seen when both the bladder and bowels are irradiated.

In spite of treatment by radical prostatectomy or radical radiotherapy, cancer cells can recur, suggesting spread of the disease. Patients whose disease recurs after curative treatment are acknowledged as being commonly understaged at diagnosis (Kirby et al, 1996). Subsequently, following restaging of the disease, hormone therapy may be offered after their initial treatment. For example, following failure of initial radiotherapy, defined biochemically as three consecutive increases in PSA levels from the PSA nadir (lowest PSA level) (American Society for Therapeutic Radiology and Oncology (ASTRO) Consensus Panel, 1997). Patients’ long-term outcome may be improved by hormone treatment afterwards, in order to prevent further disease progression such as spread to bone or lymph nodes. This approach is referred to as adjuvant-hormone therapy. However, the optimal duration and timing of adjuvant-hormone therapy remains unresolved (Soloway, 1998).

An additional method of treating prostate cancer which is gaining popularity is a procedure called brachytherapy. ‘Brachy’ (therapy) comes from the Greek -word meaning ‘short’ and is used to describe a treatment where radioactive sources or materials are placed in or close to the tumour. This technique involves placing radioactive ‘seeds’ into the prostate. Evidence from a number of clinical studies suggests that adjuvant hormone therapy with an LHRH may be an advantage only for patients with high risk of disease spread outside the prostate (D’Amico et al, 1998; Merrick et al, 2003). Where the prostate gland is over 60cc in volume, hormone treatment may also be used for 3-6 months to reduce the size of the prostate, making it easier to insert the needles and reduce the number of seeds required (Nag et al, 1999), although it remains unclear as to which form of hormone treatment should be used (Lee, 2002).

Locally advanced prostate cancer

The term ‘locally advanced’ prostate cancer refers to the disease that is no longer confined to the prostate, that has started to invade nearby organs such as the bladder and seminal vesicles, but where there is no evidence of spread either to the lymph nodes or other sites such as bone (Kirby, 2002). For patients with locally advanced disease, the impact on quality of life is likely to be an important consideration as therapy may be continued for several years (Anderson, 2003).

Table 4. Common anti-androgen treatments for prostate cancer

Hormonal treatment followed by surgery

As already discussed in the context of localized prostate cancer, hormone therapy can be used as neoadjuvant or adjuvant, often by the administration of an LHRH analogue together with an anti-androgen to prevent androgen stimulation of the tumour. This treatment has been shown to reduce prostate volume and tumour volume; however, it has not yet been shown to improve overall survival (Bono, 2004).

Radiotherapy followed by hormone treatment

Adjuvant therapy with a LHRH analogue, started at the beginning of external beam radiotherapy treatment and continued for 3 years, has been demonstrated by Bolla et al (1997) to improve overall survival m a clinical trial of patients with locally advanced prostate cancer.

Hormone treatment alone

The development of LHRH analogues and non-steroidal anti- androgens has produced treatments that reduce the intraprostatic concentration of DHT by over 80% (Bono, 2004). This is comparable to the reduction that can be achieved by surgical orchidectomy (Kirby et al, 2001). However, the psychological implications of the removal of both testes must be considered. Many patients find the concept of surgical removal worrisome and distasteful and may prefer treatment with an LHRH analogue to surgery (Kirby et al, 1996).

Figure 3. Mechanism of action – steroidal anti-androgens; LH = luteinizing hormone.

With the use of an LHRH analogue, the initial increase in testosterone can cause a tumour flare phenomenon, which can exacerbate existing symptoms, leading to urinary problems, such as decrease in urinary flow and urinary retention, or spinal cord compression (Coptcoat, 1996). Testosterone levels reach their lowest levels after about 21 days of the LHRH analogue being administered. An anti-androgen should therefore be given before and during the first 2 weeks of therapy to suppress these potential effects (Aus et al, 2003).

Monotherapy with the anti-androgen bicalutamide at 150 mg/day has been shown to be just as effective in locally advanced disease as treatment with orchidectomy or the use of an LHRH analogue (Anderson, 2003). The advantage of this option is that sexual interest and function may be preserved. Some men may understandably opt for the treatment that has a lesser impact on this important aspect of their lives.

Intermittent hormone therapy

There is some evidence to suggest that continuous androgen deprivation therapy may in fact increase the rate of progression of prostate cancer to a point -where it is no longer sensitive to androgen deprivation and therefore the prostate cancer becomes resistant to hormone therapy sooner – this is known as ‘hormone escape’ (Kirby et al, 2001). Attention is now being focused on the use of LHRH analogues for an initial 36 weeks, after which, providing the PSA is within the normal range at 32 weeks, the treatment is discontinued (Kirby et al, 2001). Such a regimen allows serum testosterone levels to return to normal, which renders the cells within the prostate more sensitive to androgen deprivation (Anderson, 2003). Therapy can be reintroduced when the PSA reaches pre-treatment levels. Studies looking at the long-term safety and effectiveness are still under way, and this approach should be regarded as experimental.

Figure 4. Mechanism of action – non-steroidal anti-androgens; LH = luteinizing hormone.

Metastatic prostate cancer

Once cancer has spread to the lymph nodes and to sites, such as bones, it is referred to as metastatic disease (Buck, 1995). Unlike early or locally advanced disease, metastatic prostate cancer is associated with high mortality within 5 years of diagnosis, with as many as 70% of patients dying from, rather than with, prostate cancer (Kirby et al, 1996).

Androgen deprivation, either by orchidectomy or treatment -with LHRH analogues, remains the mainstay of treatment as a palliative approach (Coptcoat, 1996). Patients with metastatic disease typically relapse within 18-24 months on the initiation of hormonal treatment (Anderson, 2003). Comparative trials have shown that the response rates obtained with LHRH analogues are comparable to those after orchidectomy, in terms of time to disease progression and overall survival (Vogelzang et al, 1995). However, despite its comparative effectiveness, orchidectomy, with the combination of the irreversible nature of the procedure, sexual side-effects, and the psychological burden associated with the removal of the testes, has been shown to be less appealing for most patients (Clark et al, 2001). However, this may be indicated for patients with poor compliance to tablets or injections, imminent spinal compression or those who object to regular medication.

Maximal androgen blockade

Although both orchidectomy and LHRH treatment produce good initial responses, it is known that the average time for disease progression is less than 18 months (Prostate Cancer Trialists’ Collaborative Group, 2000). One factor that may contribute to this poor prognosis and outcome is the continuing secretion of adrenal androgens. This has led to the use of both an LHRH analogue and an anti-androgen to provide maximal androgen blockade (MAB). A meta- analysis of 27 studies comparing MAB with LHRH or orchidectomy alone, has shown a small difference in survival favouring MAB (25.4% vs 23.6%) (Prostate Cancer Trialists’ Collaborative Group, 2000). Kirby et al (2001) suggest that such treatment should be considered in younger and fitter patients who are most likely to die from prostate cancer itself rather than from some comorbid condition.

Figure 5. Mechanism of action: oestrogens. LHRH = luteinizing hormone-releasing hormone; LH = luteinizing hormone.

Androgen-independent disease

In almost all cases, advanced prostate cancel-treated by any form of androgen therapy will eventually, when the prostate cancer cells become less sensitive to hormonal control, begin to grow again – a phenomenon known as ‘hormone escape’ or ‘an\drogen independence’ (Bono, 2004). Prostate cancer cells are heterogenous in their sensitivity to androgens. This means that the cells can range from being androgen-dependent, through androgen-sensitive to androgen- independent (Kent and Hussain, 2003). Failure of hormone therapy is detected by a rising PSA, and thus an increase after initial successful androgen treatment almost inevitably indicates disease progression (Aranha and Vaisarnpayan, 2004). Although the prognosis is poor, it is generally accepted that once the cancer has become hormone resistant, treatment to deprive the prostate cancer cells of androgens should be maintained with second-line therapy, to delay disease progression. Paradoxically, patients who have progressed on MAB may have a response if the anti-androgen is withdrawn (Mason and Moffat, 2003).

Table 5. Common effects of hormonal treatment

The synthetic oestrogen diethylstilboestrol (stilboestrol) is used in this manner, but side-effects such as gynaecomastia, deep- vein thrombosis and other cardiac complications, limit its use as first-line therapy (Malkowicz, 2001). This treatment is often used in combination with aspirin to reduce thrombosis and risk of cardiovascular toxicity. Response rates of 30-35% have been seen in reducing disease progression (Newling, 1997).

At this stage of the disease, perhaps more so than at any other time, any treatment is palliative and is an attempt to prolong life. Such treatments must therefore be individualized and be judged in their use against the remaining quality of life of the patient.

The effects of hormone treatment

In response to the treatment of prostate cancer, patients’ reactions will vary considerably. Disturbing the body’s balance of sex hormones can lead to various undesirable and upsetting effects. Patients with advanced disease may often be asymptomatic. It is therefore essential to counsel the patient regarding the potential impact of this therapy on his quality of life. The most common effects of treatment are outlined in Table 5.

Key nursing roles

Men diagnosed with prostate cancer often feel surprised, angry, confused and depressed. Feelings may change from hope and courage to despair and fear, and back again (Kunkel et al, 2000). With increasing numbers of men being diagnosed with this disease, it is essential that they are fully informed about treatments and the inevitable effects on their quality of life. Central to this must be their involvement in the decision-making process to guide them through what may seem a very uncertain and complicated patient journey. Support for the family as a supporting unit is also critical.

With the proliferation of so many nurse-led initiatives emerging in urological cancer care, many nurse specialists are at the forefront of discussing, administering and reviewing the effects of hormone therapy to patients with prostate cancer, as part of their own clinical patient caseloads. It is essential that in this arena, information about the condition and the effects of treatment are discussed with the patient, to empower him to make a decision based on his priorities, the disease, and knowing the potential effects that the treatment may have on his psycho-social-sexual wellbeing. Where treatments may have a lesser effect on erectile function, irrespective of the patient’s age, the potential for these to be used should be discussed.

With many nurse-led clinics reviewing such patients on a continual basis, it is often the nurse who will be more aware of any changes in a patient’s condition, adverse effects of treatment and changes in response to treatment. The nurse is in a key position to assess, plan and implement care to address such issues that may improve the quality of life of the patient. As the disease will invariably progress, the need for palliative care may ensue – the nurse is ideally positioned to discuss such issues -with the patient and his family and to refer patients for ongoing community support, which may lessen the need for repeated and sometimes distressing hospital admissions. There is also a key role to educate other nurses regarding these treatments and how they should be administered.

Conclusion

In prostate cancer, patients may present with many concerns about the effect of treatment on their quality of life, for a condition that may not even be symptomatic. In considering hormone therapy treatment, patients need to be aware of the overall benefits, in terms of survival advantages where the disease is incurable and how the treatment may affect them in the long term. Nurses are in an ideal position to discuss such issues with patients and they should be an integral part of the patient journey from the point of diagnosis.

KEY POINTS

* Prostate cancer is the most common male cancer in the UK with over 27000 cases and over 10 000 deaths per year, with numbers increasing annually.

* Androgens are fundamental to the continuing growth of prostate cancer cells, with treatment strategies aiming to block their effects or decrease their production.

* Patients with prostate cancer may often be asymptomatic.

* The effects of hormone therapy must be discussed and considered with the patient.

* The patient’s journey is often uncertain and key to this is patient empowerment through knowledge and support, to guide him through the decision-making process.

* The nurse is ideally situated to administer hormone therapy, assess the effectiveness of treatment and the patient’s wellbeing when the disease progresses.

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Lawrence Drudge-Coates is Macmillan Urology Clinical Nurse Specialist, Department of Urology, King’s College Hospital, London

Accepted for publication: February 2005

Copyright Mark Allen Publishing Ltd. Apr 14-Apr 27, 2005

Cocaine Can Harm Heart’s Blood Vessels

High incidence of aneurysms found in users, study finds

HealthDay News — Cocaine users seem to have an unusually high incidence of coronary artery aneurysms, weakened areas of heart blood vessels that raise the risk of heart attacks, new research finds.

The study included 191 men and women in their 40s who had angiography, an X-ray of blood vessels, because of known or suspected heart disease. Aneurysms were found in 34 of the 112 persons who reported using cocaine and only six of 91 nonusers.

The study, by physicians at the Minneapolis Heart Institute Foundation, appears in the May 10 issue of Circulation.

That higher incidence of aneurysms may help explain why cocaine users have been found to have a high risk of heart attacks, said Dr. Timothy D. Henry, director of research at the foundation.

Henry suggested two possible explanations for the increased incidence of aneurysms.

“Cocaine use causes periodic hypertension, periods when the blood pressure goes up sharply,” he said. “Having such episodes of high blood pressure over the course of time can lead to formation of aneurysms.”

Cocaine is also known to damage the endothelium, the delicate lining of blood vessels, which could contribute to the weakening of the arteries, Henry said.

Whatever the explanation, the report is “another reason to tell people how dangerous cocaine is,” he said.

The study cited estimates by the federal Substance Abuse and Mental Health Services Administration that 27.7 million Americans — 12 percent of those 12 and over — had used cocaine at least once in 2001, and that 1.7 million had used it in the previous month.

Most studies of cocaine and heart damage have concentrated on immediate problems, Henry noted. The new study raises the possibility that even short-term use can cause damage decades later, he said. One man in the study who was found to have an aneurysm said he had used cocaine heavily for a two-year period 15 years earlier, Henry said, so the drug “can cause long-term damage that you have to live with the rest of your life.”

Dr. Murray Mittleman, director of the Cardiovascular Epidemiology Research Unit at Beth Israel Deaconess Medical Center in Boston, said the new study “provides clues to the mechanisms of heart attacks occurring in people who are cocaine users.”

“It is an important step forward in understanding the biology of what happens in cocaine use,” Mittleman said.

But follow-up studies are needed because of the way the study was carried out, he said.

“They didn’t start out looking at people who used cocaine,” Mittleman said. “They looked at people who had angiography for some clinical reason. It is possible that this is a special group of cocaine users.”

Nevertheless, the report “gives some insight into why we observe a higher rate of cardiac problems in cocaine users,” he said.

More information

Minneapolis Heart Institute Foundation

For more on cocaine’s risks to the heart, visit the American Heart Association.

Why Do I Keep Perspiring so Much?

Q: I’m 16 and for the past two years I’ve been having problems with underarm perspiration.

I can’t wear what I want because of the sweat patches and it is really affecting my confidence. I have tried every deodorant going but nothing works. A friend told me that Botox may help. Do you advise this? And do you have any other suggestions?

A: This can be an embarrassing problem and I understand how it affects your self-confidence. Let me explain a little bit about the biology first. There are two types of gland that produce discharge: endocrine and exocrine.

Endocrine glands release hormones, which are transported to different cells of the body. We are concerned with the exocrine glands, which produce discharge on surfaces that are exposed to air. These include the skin, eyes, lungs and gut among others.

There are four types of exocrine gland, of which the apocrine glands concern you most. These are targeted by the deodorant and antiperspirant industry.

They’re found in the armpits, around nipples and in the groin. Because they communicate through ducts linked to the hair follicles, they begin to release a discharge at puberty when hair grows in these areas.

Apocrine discharge is sticky, cloudy and relatively thick. It is also potentially odorous, which may be due to diet, pharmaceutical drugs and some supplements (such as vitamin B complex). The problem is made worse because perspiration is a rich source of nutrients for bacteria, so they cluster around it and create a smell as they decompose.

What’s more, the activity of these gland cells is an involuntary reaction.

In some cases, the warm moisture in these areas can encourage a bacterial or fungal infection; the body tries to counter this with a thicker, disinfectant type of discharge more perspiration. If this happens, the smell may get worse and the thick discharge ‘rots’. So it is essential that people with perspiration problems shower thoroughly twice a day.

The other type of sweat gland, merocrine, is distributed all over the body, particularly the palms and soles of the feet: some sufferers often find their hands and feet perspire profusely. This can be uncomfortable but it is a more benign discharge, as it is 99 per cent water, which is odourless and also helps to cool the body and discharge toxins.

Your excessive sweating may be caused by a combination of factors, including nervousness or anxiety, which causes tightness of muscles and tends to make circulation sluggish; overheating of the body (because you have eaten chillies, for instance, or had a hot drink, or your bedclothes are too heavy), and possibly your diet.

You can have surgery for this condition or Botox injections, and I advise you to talk to your doctor about the options. However, before embarking on more drastic measures, I suggest that you follow this noninvasive regime.

Neck massage

The higher centre for the parasympathetic nervous system (which controls gland activity, among other things) and the thermostat for regulating body temperature are located in the hypothalamus in the brain.

Massaging the neck can improve blood flow to that area, enhancing the function. Ask someone to massage your neck and shoulders in the evening, a couple of times a week for ten minutes. Details are on my Lifestyle DVD (Integrated Health Group, Pounds 19.99).

Diet

In general, avoid all processed or prepared foods and eat fresh, simple food with lots of vegetables and fruit, preferably organic. Particularly avoid yeast products, canned or cured foods, red meat, pork, very spicy food, fungal cheeses (eg, blue cheese), vinegar, mushrooms, ginger, garlic, alcohol, coffee. These can all overheat the body and cause sweat to smell.

Include cooling foods such as live organic natural yoghurt, buttermilk, cucumber, squash, spring onions, courgettes, marrow, kiwi, parsley, mint, camomile, melon and summer berries (buy frozen in winter).

Treatments

Use antiseptic neem oil (available from health food stores) or Alive Skin Oil (Pounds 6 for 20ml), which also contains neem, to massage the underarms at bedtime.

Wash in the morning with mild glycerine or baby soap.

Complementary therapies

Consult a qualified homoeopath or acupuncturist.

Breathing

In general, stay calm and don’t rush. Whenever you feel nervous or the sweating starts, practise this simple breathing exercise. Breathe in for three seconds, hold your breath gently for three, then breathe out slowly for six seconds. Practise this for five minutes daily until you can do it automatically.

Clothing

Synthetic fabrics trap heat, so wear light layers in natural fabrics such as cotton or linen.

Contact information

If you have a health query you would like addressed on this page, please write to Dr Ali at YOU, Northcliffe House, 2 Derry Street, London W8 5TS, or email him at [email protected]. Dr Ali regrets he cannot enter into personal correspondence.

Always consult a qualified medical practitioner if your symptoms persist Previous health queries To see all Dr Ali’s previous YOU columns, visit his website, www.drali.com Where to buy Products, Dr Ali’s books and DVD are available from the Integrated Medical Centre shop, 43 New Cavendish Street, London W1G 9TH, tel: 020 7224 5141 (clinic, tel: 020 7224 5111). Postage and packing is charged.

Dr Ali’s books are also available from the YOU Bookshop, tel: 0870 162 5006, or visit www.you-bookshop.co.uk

Kenyan TV Report Says Herbal Medicine “Gaining Popularity” in Country

Text of report by Kenyan KTN TV on 7 May

[Presenter Michael Oyier] Tonight on Health Digest the debate over whether to incorporate herbal treatment into public hospitals has led to heated debate between doctors and herbalists. But whatever the case, herbal medicine is gaining popularity, both locally and internationally. On Health Digest tonight, KTN’s Mwendwa Kingora takes a look at why more people are turning to nature for a cure.

[Kingora] Barely a decade ago, few people would want to be associated with herbal medicine. Consulting a herbalist was equated to visiting a witch doctor and the conventional bark of trees and concoctions in bottles did not make it attractive. But all that is changing now. Herbal medicine is quickly gaining popularity as it is no longer dished out as roots or leaves. But it is moving with the times and packaged more attractive capsules, tablets, cream or powdered form.

At the Makini Herbal Clinic in Ngara [Nairobi], the practitioners have gone a step further and incorporated state of art equipment in their laboratory to diagnoze and best treat symptoms presented to them.

[Dr Sizyah Kimambo, herbal practitioner] So when we decided to bring up the machines for scanning and lab facilities it was because most of them, when they come here, though they come with chronic diseases, they believe that if we do the examination ourselves they tend to trust us more.

[Kingora] One of the most sought-after natural medicine is the Muarubaini, the one that is said to treat over 40 ailments. It has immune boosting characteristics and is said to manage rheumatism, diabetes, asthma and kidney disorders. Renowned herbalist Dr [Helge] Linck maintains that he can heal diseases that conventional medicine has failed to handle.

[Linck] We are not allowed to say they can cure asthma but we get rid of it and I have got hundreds and thousands of letters from people who had asthma terribly for five, 10 years and after one or two weeks they don’t need to take the inhaler. And now they are all right. Or if you got tonsillitis, even if the doctor has said, ‘look here, you come back in two or three days time they will operate your sinus’, give me two days or three days, you take my medicine, when you go there, I guarantee you that – your tonsils all right we don’t need to operate. What have you taken?

[Kingora] He says the human body works in harmony with nature.

[Linck] To find out why is your immune system not doing what it should do, that is to fight your asthma, your allergy or your cancer and all that there, and the usually within 20 or 30 minutes I know that is the problem. Now then we give your body what is needed so that the immune system will work 100 per cent and get rid of it. So it is not me, it is the medicine or your body, which does it.

[Kingora] Those who opt for herbal medicine are full praise for the treatment, most of them having been diagnosed by conventional doctors but visit these clinics as a last resort.

[David Wambua, patient, in Swahili] I was suffering from ulcers. I have been to an hospital in Kitengela [southeast of Nairobi] where I was tested. The drugs I was asked to buy did not help. I went back to Machakos, where I was tested again and asked to buy more drugs. I’m yet to feel better. I kept on falling sick every two months.

[Kingora] The World Health Organization has also recognized the importance of alternative medicine and is recommending that it is incorporated into public hospitals to manage diseases and poverty. But the debate between the two institutions rages on.

[Dr Davy Koech, director, Kenya Medical Research Institute] The institute itself is keen in developing this area. It is also keen in exploring the biodiversity that is available in order to preserve what is there.

[Linck] If we wanted to help the nation, we could do a lot of good ourselves. But I know that the pharmaceutical companies are very powerful and very rich and they don’t like persons like me.

[Kingora] The medicinal value of herbs is not in question. For centuries, herbal medicine has been used to cure various ailments.

But conventional doctors now want a policy framework that will regular herbalists.

COSMETIC SURGERY AND THE Cultural Construction OF Beauty

Throughout history, certain members of nearly all cultures have deliberately altered their body’s natural appearance. Padaund women use rings to elongate their necks, Victorians constricted their waists with corsets, and other cultures have practiced foot binding. When we look closely at each activity, we quickly uncover the junction of ideology of beauty held by members of these societies and the technology available to achieve that ideal.

Today, we live in a time when medicine can cure the body and also reshape it. Hence, many people use biomedical means, such as steroids and hormones to alter their bodies. Additionally, cosmetic surgery is becoming increasingly available and affordable to people of all ages, including teenagers. According to journalist Alissa Quart (2003), “Teenagers now alter their bodies extremely and proudly” (p. 115). She reports, from 2000 to 2001, the number of cosmetic surgeries on teens ages 18 and under rose nearly 22%.

In this article we explore the growing popularity of cosmetic surgery. Our work is inspired and informed by many of the articles contained in the March 2003 issue of ‘ArL Education, which encourage art educators to address visual culture (Villeneuve, 2003). Specifically, we adopt an instructional approach to our subject as advocated by Karen Keifer-Boyd, who recommends we begin with what is meaningful in our lives today and then look for representations of those issues in visual culture (Keifer-Boyd, Amburgy, and Knight, 2003, p. 48). We draw examples from reality television, advertising, dolls, and films made for children. Next, we examine the work of Orlan, a performance artist who repeatedly underwent cosmetic surgery in a quest to challenge dominant standards of beauty. By discussing examples from both popular culture and fine arts, we hope to achieve the goals of art education as posited by Arthur Efland (2004) who writes that art education should provide “the freedom to explore multiple forms of visual culture to enable students to understand social and cultural influences affecting their lives.” (p. 250).

The Construction of Beauty in Visual Culture

Myths and beliefs about beauty are deeply embedded in our culture and are transmitted from early childhood onward. Consider how fairy tales, movies made for children, and dolls reinforce the notion that beauty is a prerequisite for happiness. Traditionally, the hero or heroine is portrayed as young, beautiful, and white while the villain is depicted as old, ugly, and dark. One such example is Disney’s Little Mermaid (1989), an animated version of the Hans Christian Andersen fairy tale about Ariel, a mermaid who has fallen in love with the handsome prince, Eric.1 Ariel makes a deal with Ursula, a squid, to trade her exquisite voice for a pair of legs in order to pursue Eric. Henry Giroux (1998), a long-time critic of the Disney corporation, points out that Ursula, the “large, oozing, black and purple squid in the Little Mermaid, gushes with evil and irony” while the mermaid Ariel appears to be “modeled after a slightly anorexic Barbie doll” (p. 58). Although there are exceptions, generally in these stories, only a beautiful princess and her prince live happily ever after.

While many dolls, such as Bratz and American Girls, may communicate standards of beauty to the children who play with them, critics who want to comment on cosmetic surgery most often cite Barbie, Mattel’s best selling doll. In one such instance, Cindy Jackson, an American talk show celebrity, had more than 20 surgical procedures in an attempt to resemble Barbie (Goodall, 1999). Much has been written about the Barbie doll, which according to Shirley Steinberg (1998) “celebrates whiteness-blond whiteness in particular- as a standard for feminine beauty” (p .217). Susan Jane oilman (2000), reflecting on her own childhood experiences of playing with Barbie, writes, “We urban, Jewish, black, Asian and Latina girls began to realize slowly and painfully that if you didn’t look like Barbie, you didn’t fit in…. You were less beautiful, less valuable, less worthy” (p. 17). oilman’s comments are particularly apt when we consider the history of cosmetic surgery.

Sander oilman (1999), in his book, Making the Body Beautiful: A Cultural History of Aesthetic Surgery, explains that the growth of cosmetic surgery coincided with the spread of so-called race science that linked one’s physical appearance to one’s temperament, character, and intelligence. For example, those considered racially inferior to the English middle class, such as the Irish, Welsh, and the lower classes, were thought to have protruding jaws. Men of intelligence were considered to be those with less prominent jaws. Some cosmetic surgeons now consider the face of Catherine Zeta- Jones, a Welsh actress, as the representation of ideal beauty. Early scientists also believed that physical and mental illness, prostitution, and signs of criminal behavior were thought to be perceptible on the exterior of the body as a symbolic reflection of one’s inner state. Early cosmetic surgery held the promise of curing one’s inner condition by transforming one’s outer appearance.

Today, advertisements frequently carry the suggestion that cosmetic surgery will enhance self-esteem and improve one’s quality of life (Sarwer & Crerand, 2004). These ads appear on television, billboards, and in magazines, where young beautiful models are frequently used to depict postoperative results. Prior to 1982, the American Medical Association prohibited all advertising for medical services (Sullivan, 2001). The Supreme Court over turned this ruling and paved the way for cosmetic surgeons to actively solicit customers. Generally, advertisements for cosmetic surgery differ from other medical ads in that they often resemble ads typically found in fashion and beauty magazines.

Deborah Sullivan (2001) regards women’s magazines as one of the most important sources of information about cosmetic surgery. She examined 171 magazine articles about cosmetic surgery from 1985 to 1995 to determine how magazines participate in the cultural construction of appearance. Sullivan (2001) found that, “Articles about cosmetic surgery in women’s magazines provide readers an opportunity to learn physicians’ ideology about the problematic nature of body parts that fall short of the ideal” (p. 156).

The many makeover-based reality shows have, in part, contributed to the demand for cosmetic surgery. In 2003, the ABC television show, Extreme Makeovers, was the second highest rated program for adults under 50 (Sarwer and Crerand, 2004). Another example, The Swan, featured 18 women who all described themselves as “ugly ducklings.” In this show each woman was given an individualized team, which consisted of a personal trainer, a therapist, a dentist, and a surgeon, to help her transform into a beautiful person. During the final episode, one woman was crowned the “Ultimate Swan.”

For a younger audience, teenagers Mike and Matt Schlepp, starred in I Want a Famous Face, an MTV cosmetic-surgery reality show. The MTV crew followed Mike and Matt through two months of countless surgeries where they spent $37,000 of their own money to look like Brad Pitt. The Schlepps, as well as the winners of The Swan and Extreme Makeover were later featured on talk shows such as Oprah, and Dr. Phil. Television, like magazines and children’s toys and movies, provides visual examples of beautiful individuals. People become dissatisfied with their appearance when they perceive a discrepancy between their actual appearance and an ideal, whether that ideal is that of a doctor, celebrity, or a toy manufacturer.

Cosmetic surgery involves aesthetic judgments, and when we closely inspect those judgments, we can see that standards of beauty are largely culturally determined. The artist Orlan, whom we discuss in the next section, critiques dominant norms of beauty. Her Reincarnation of St-Orlan specifically comments on current cosmetic surgery practices.

Challenging Dominant Standards of Beauty Through the Art of Orlan

The French artist Orlan is regarded as one of the more important artists of the late 20th century. She uses her face as her primary medium and cosmetic surgery as her sculpting method. On May 30th, 1987-her 40th birthday-Orlan embarked upon a project that married cosmetic surgery with performance art. The Reincarnation of St- Orlan, which entailed nine separate operations, involved re- sculpting (Man’s face according to ideals of female beauty established by male artists throughout history. Orlan first created a computer-generated self-portrait based on features taken from women in famous artworks. Her forehead, for example, was taken from Da Vinci’s Mona Lisa; her chin from Botticelli’s Venus, and her mouth from Boucher’s Europa (Ince, 2000, p. 6). Orlan then turned to cosmetic surgery to sculpt her face to match the computer-generated image.

Orlan’s art is not the completed operation, but rather the process of the operation, during which she remained fully conscious and in communication with her remote audience of gallery visitors via satellite (Goodall, 1999). Orlan talked animatedly to her audience or read to them from texts chosen to serve as commentaries on what is taking place. One key text read by Orlan durin\g her performance was from Lacanian psychoanalyst Eugnie Lemoine- Luccioni’s book (1983), LaRolte (The Dress). LemoineLuccioni, who devotes a chapter to Orlan’s early performances, writes poetically about the discrepancy between the way in which one appears versus one’s own self-perception. She states, “Skin is deceptive… I have black skin, but I am a White person; the skin of a woman but I am a man. I never have the skin of what I am” (Lemoine-Luccioni, 1983, p. 95, translation). Although Orlan’s first performances were concerned with ideals of Western beauty, the seventh, eighth, and ninth operations involved placing implants into her upper cheeks and the sides of her forehead to give the impression of budding horns (Goodall, 1999). After the seventh operation, Orlan displayed 41 self-portraits consisting of photographs taken on the day the surgery was performed, and on each subsequent day after until the healing process was complete (Moos, 1996).

Orlan’s work has been sensationalized by the media, and critics often write that she is uncritically accepting socially imposed ideals of beauty by using cosmetic surgery to become the “ideal woman” (Hirschhorn, 1996, p. 117). Moreover, according to Ince (2000), “Orlan has been called a publicity freak and a surgery junkie, and had her mental equilibrium repeatedly called into question” (p. 45). Others hail her work as a radical feminist critique of beauty practices. Anthony Shelton (1996), for example, writes that Orlan’s goal is to spare women from cosmetic surgery by showing its graphic and horrible side (p. 107). Orlan, however, states, “My work is not a stand against cosmetic surgery, but against the standards of beauty, against the dictates of a dominant ideology that impresses itself more and more on the feminine flesh” (Brand, 2000, p. 293). Davis (1997) posits that for Orlan, cosmetic surgery is a way for women to regain control over their bodies, but she believes Orlan’s intention was never meant to reflect the surgical experiences of “ordinary” women (p. 176). Davis conducted in-depth interviews with women who had or planned to have cosmetic surgery and found that the women did not have surgery to become more beautiful. Davis writes, “Cosmetic surgery was an intervention in identity. It enabled them to reduce the distance between the internal and external so that others could see them as they saw themselves” (p. 175). Others, such as Naomi Wolf (1991), are not as optimistic about women’s motivation to have surgery. She posits that women are pressured to have cosmetic surgery in order to obtain the ideals promoted by the male-dominated fashion industry and its advertising campaigns. Moreover, while more women than men have cosmetic surgery, the number of men having surgery is increasing (Sarwer & Crerand, 2004).

Technology has given us safer, less invasive surgical procedures, and advertising has increased media awareness about those procedures. We are witnessing a greater willingness of people to have surgery to enhance their appearance, in reality as well as on reality television. There is no consensus on the meaning of enhancing the body. Some condemn it, while others celebrate it. We may hold strong opinions, but like KeiferBoyd (2003) we believe our role as art teachers is to guide students to construct their own meanings by critically examining how their immediate concerns are represented by others, particularly through mass media and popular culture, and contemporary art.

Reconstructing Notions of Beauty in the Art Classroom

Art educators Charles Garoian and Yvonne Gaudelius (2001) argue that teachers can use the work of Orlan as a critical metaphor to question the aesthetic impact of technology and to critique the discourse that normalizes cosmetic surgery. They write, “The rigorous documentation of her surgeries and her performances of canonical ‘beauty’ function technologically to enable us to question various ways in which the body makes and carries meaning” (p. 342). Orlan acknowledges the ways in which our choices about appearance are social and historical (Pitts, 2003) and may share common ground with teenagers who are exploring and experimenting with their identity through fashion, tattooing, and piercing. Admittedly, Orlan’s work is provocative and not all teachers will be comfortable discussing her work. However, discussions around Orlan’s art can address questions raised by Peg Zeglin Brand (2000), in the introduction to her book, Beauty Matters. She asks, “What is beauty and how does it operate within the context of our particular culture? What are the ideals of feminine beauty and are they relevant to portraying beauty in art?” (p. 4).

Teachers can help students carefully examine “before and after” images used in cosmetic surgery and consider how these images rely on “photographic truth.” Sturken and Cartwright’s (2001) book, Practice of Looking: An Introduction to Visual Culture, is particularly helpful to teachers. Their work can enable teachers to lead discussions on the role these photographs play as scientific evidence. Sturken and Cartwright (2001) write, “The photographic image has often been seen as an entity stripped of intentionality, through which the truth can be told without mediation or subjective distortion” (p. 280). As viewers, we are apt to accept an image as reality, since as Sturken and Cartwright assert, “Scientific imagery often comes to us with confident authority behind it… we often assume it represents objective knowledge” (p. 279). Through the use of “before” and “after” images, the media attempts to provide scientific evidence that surgery has not only improved appearance, but has provided happiness. Students can be led through discussions to see that “before and after” images, like other visual images, are in need of interpretation.

According to Barrett (2003), “If the messages carried by visual culture are not interpreted, we will be unwittingly buying, wearing, promoting, and otherwise consuming opinions with which we may or may not agree” (p. 12). Advertisements for cosmetic surgery images carry ideology, but may appear neutral. We asked students enrolled in a course on visual culture to collect and discuss ads for cosmetic surgery. Using a variety of ads, students pointed out that reproductions showing people prior to surgery were dimly lit, whereas “after” images were brighter. Others noted that “after” images showed people with better posture and with smiling faces. One student located an ad in which a woman of color was shown with lighter skin after her surgery, even though the ad was for a liposuction procedure. We also viewed and discussed how art and aesthetics were used to advertise cosmetic surgery. A number of ads, like Orlan, appropriated images from Renaissance paintings, such as Botticelli’s Venus. Elevate, a magazine devoted entirely to cosmetic enhancement, proved to be good source for images that demonstrate the symbiotic relationship between art and cosmetic surgery.

Sturken and Cartwright remind us that all ads are about transformation in that they are designed to offer people who are in some way dissatisfied with a product that will alleviate their dissatisfaction. According to Sturken and Cartwright (2001), ads offer “figures of glamour that consumers can envy and wish to emulate, people who are presented as already transformed, bodies that appear perfect and yet somehow attainable” (p. 213). Orlan’s work is in direct contrast to the “before-and-after” images of people who have undergone cosmetic surgery used so pervasively by media. Orlan’s photographs of her bruised and swollen postoperative face highlight the process of cosmetic surgery and not its end result. When examining advertisements, television, or the work of Orlan, students can consider ways in which changing one’s appearance changes one’s identity. “Where is your identity located? Is it visible or invisible?”

With younger students, we can examine notions of beauty prevalent in popular culture, starting with fairytales. Many cosmetic surgery based reality shows are updated versions of fairy tales that offer magical and instant transformation. Books such as Robert Munsch’s (1980) The Paper Rag Princess provide an alternative to the typical tale. Munsch’s book begins with the usual tale of Elizabeth, “a beautiful princess, who lived in a castle, had expensive princess clothes, and was going to marry a prince named Ronald” (p. 2). Her plans are changed after a dragon smashes her castle, burns her clothes, and carries off Prince Ronald. In Munsch’s tale, however, it is Elizabeth who cleverly outsmarts the dragon and rescues Ronald. When the prince rejects Elizabeth because of her dirty clothes and messy hair, she leaves him, dancing off into the sunset, joyfully and alone. The film, Shrek (2003), also parodies traditional fairy tales. Stories such as this inform students that women should not feel that the only quality they have to offer is their appearance, or that it should be the primary means through with they will be evaluated by others.

Conclusion

We echo Orlan and do not take a stand against cosmetic surgery. We wish to help students consider how their notions about beauty are socially constructed and to provide them with tools for promoting the respect and appreciation of inner qualities and individual strengths. We are aware, however, that for those students who are teased unmercifully by classmates, cosmetic surgery holds great promise to end their suffering. We also do not want to alienate students for their choice to elect to have cosmetic surgery. Perhaps, in the near future, cosmetic surgery will be considered as common as cutting or dying one’s hair or wearing make-up. On the other hand, it may be regarded with contempt, in the same manner as foot binding. Regardless, our intent is to help students critically examine the messages put forth by popular culture, as well \as the fine arts, and to make informed choices to accept or reject those messages.

People become dissatisfied with their appearance when they perceive a discrepancy between their actual appearance and an ideal, whether that ideal is that of a doctor, celebrity, or a toy manufacturer.

Through the use of “before” and “after” images, the media attempts to provide scientific evidence that surgery has not only improved appearance, but has provided happiness. Students can be led through discussions to see that “before and after” images, like other visual images, are in need of interpretation.

ENDNOTES

1 The authors thank reviewer Deborah SmithShank for her suggestion to use the example of Disney’s Little Mermaid(TM) to help “make the case for cultural icons of beauty and ugliness.”

REFERENCES

Barrett, T. (2003). Interpreting visual culture. Art Education 56(2), 6-12.

Brand, RZ. (2000). Beauty matters. Indianapolis: Indiana University Press.

Davis, K. (1997). ‘My body is my art’: Cosmetic surgery as feminist Utopia? In K. Davis (Ed.) Embodied practices: Feminist perspectives on the body (pp. 169-181). London: Sage Publications.

Efland, A. (2004). The entwined nature of the aesthetic: A discourse on visual culture. Studies in Art Education, 45(3), 234- 251.

Garoian, C. & Gaudelius, Y. (2001). Cyborg pedagogy: Performing resistance in the digital age. Studies in Art Education, 42(4), 333- 347.

Gilman, S.J. (2000). Klaus Barbie and other dolls I’d like to see. In Ophira Edut (Ed.). Body Outlaws. New York: Seal Press.

Gilman, S. (1999). Making the body beautiful: A cultural history of aesthetic surgery. New Jersey: Princeton University Press.

Giroux, H. (1998). Are Disney movies good for your kids? In S. Steinberg and J. Kincheloe, (Eds.), Kinderculture: The corporate construction of childhood. Boulder, Colorado: Westview Press.

Goodall, J. (1999). An order of pure decision: Un-natural selection in the work of Stelarc and Orlan. Body and Society, 5(23), 149-170.

Haiken, E. (1997). Venus envy: A history of cosmetic surgery. Baltimore: Johns Hopkins University Press.

Hirschhorn, M. (1996). (Man: Artist in the post-human age of mechanical reincarnation: Body as ready (to be re-) made. In Griselda Pollock (Ed.), Generations and geographies in the visual arts: Feminist readings. New York: Routledge.

Ince, K. (2000). Orlan: Millennial female. New York: Berg.

Keifer-Boyd, K., Amburgy, P., & Knight, W. (2003). Three approaches to teaching visual culture in K-12 school contexts. Art Education, 56(2), 44-51.

Lemoine-Luccioni, E. (1983). La robe: Essai psychanalytique sur le vtements. Paris: ditions du Seuil.

Moos, D. (1996). Memories of being: (Man’s theater of the self. Art + Text, 54, 67-72.

Munsch, R. (1980). The paper bag princess. Toronto: Annick Press, Ltd.

Pitts, V. (2003). In the flesh: The cultural politics of body modification. New York: Palgrave Macmillan.

Quart, A. (2003). Branded: The buying and selling of teenagers. Cambridge, MA: Perseus Publishing.

Sarwer, D., & Crerand, C. (2004). Body image and cosmetic medical treatments. Body Image, 7(1), 99-111.

Steinberg, S. (1998). The bitch who has everything. In S. Steinberg and J. Kincheloe, (Eds.), Kinderculture: The corporate construction of childhood. Boulder, CO: Westview Press.

Shelton, A. (1996). Fetishism’s culture. In N. Sinclair (Ed.), The chameleon body. London: Lund Humphries.

Sturken, M., & Cartwright, L. (2001). Practices of looking: An introduction to visual culture. Oxford, UK: Oxford University.

Sullivan, D. (2001). Cosmetic surgery: The cutting edge of commercial medicine in America. New Brunswick: Rutgers University Press.

Villeneuve, P. (2003). Editorial. Art Education, 56(2), 4-5.

Wolf, N. (1991). The beauty myth: How images of beauty are used against women. New York: William Morrow.

Lorrie Blair is associate professor in the Department of Art Education, Concordia University, Montreal.

E-mail: [email protected]

Maya Shalmon is a graduate student at Concordia in Creative Arts Therapies.

E-mail: [email protected]

Copyright National Art Education Association May 2005

Wellness Center Offers Myriad Programs

Tesuque Pueblo members have a place to grow — a place to join together in various social, recreational, wellness and spiritual aspects.

The Taytsugeh Oweengeh Intergenerational Center, which opened last November, cost

$4.6 million to build and offers all tribal members an array of activities, from a senior center with diabetes and nutrition programs to a full gymnasium, weight room and recreation area as well as an after-school program for the youth.

“The center was built to promote health and fitness, and to bring the communities together,” said Dolly Narang, the center’s executive director.

About 40 to 45 people use the facilities daily, but she would like to see more people from the community take advantage of the center. This 23,000-square-foot building, visible from U.S. 84/285, was built using profits from Tesuque’s Camel Rock Casino.

The center is open to Tesuque tribal members and employees. And programs such as basketball and volleyball leagues that could bring in more people, including nontribal members, have been discussed.

Francisco Garcia, a personal trainer and sports specialist, hopes to organize some of these leagues, possibly in a month. “It’s a beautiful facility that we have here,” Garcia said. “They are making big leaps and bounds. It has a lot to offer here.”

“Hopefully, more people here will take advantage of this,” he added.

Garcia said his job includes giving people personal fitness plans and diets to keep them healthy and to give them “a sense of pride.”

One of the most important programs at the center is its Diabetes Prevention Program, where people are screened for high blood-sugar levels and educated about diabetes, he said. Then they are directed toward a diet and fitness program to help control the disease.

Diabetes is the fifth deadliest disease in the United States and has reached epidemic proportions among Indians, according to the American Diabetes Association.

While exercise is stressed, Narang said, the center is also planning to construct a hydrotherapy pool and treatment area. She said hydrotherapy is the use of water to treat certain diseases, specifically diabetes and arthritis. “Hot-water treatment really improves strength, flexibility and the range of motion,” she added.

Narang hopes construction on the new unit will be completed by the end of the year. The pueblo also received a portion of the funding for the construction project from the Legislature.

Meanwhile, the center’s senior nutrition program provides elders 55 and older an opportunity to eat free nutritious breakfasts and lunches. Narang said the center monitors the seniors’ eating abilities and brings them into a social environment that enhances their food intake. The center also provides transportation to and from the center for these individuals.

Besides the senior program, the learning and resource center allows students to use its computers after school and to work with tutors on homework.

Before the center was built, Narang said, some of the pueblo’s youth had no place to go afterschool or on weekends. With the center, they “maintain relationships and work together,” she said.

A teen night is held each Tuesday evening, and the pueblo’s youth organize dances and other fitness activities.

Also, on May 11, a Women’s Health Day is scheduled at the center. In addition, the center is organizing a Just Move It campaign, which will encourage people to walk for exercise.

In September, the second annual Camel Rock Run is scheduled.

(Sidebar)

The center is open from 6 a.m. to 9 p.m., on Fridays from 6 a.m. to 7 p.m. and Saturdays from 7 a.m. to 4 p.m. Monday through Thursday. The center is closed on Sundays. For more information, call 955-7773.

To get more information on upcoming basketball or volleyball leagues, call Francisco Garcia at 955-7785.

Showered With Blessings; Birth Circles Give Expectant Moms Spiritual Support

Ever since the Magi delivered those epochal baby gifts, the tradition of celebrating birth with a shower of presents has been evolving. Along the way from myrrh to Tommy Hilfiger layettes, the tradition has lost some of its soul.

Today, a baby shower is as much commercial venture as social ritual. And with 21 percent of all babies in this country delivered by caesarean section last year, childbirth itself has become a medical procedure, detached from what some mothers say can be a transcendent spiritual experience.

In an attempt to restore some of the grace, an alternative pre- partum ceremony is emerging. Called a “birth circle” or “blessing way,” it has its roots in Native American culture.

Gifts not from a store

So it was that on a Saturday afternoon in mid-March, about 25 of Katie Sarnoff’s close women friends and relatives gathered in her chiropractic office in Vineland to bless the mother and her nearly there baby.

Sarnoff, 29, stood at the top of the stairs greeting her guests in a lullaby-soft voice and peaceful yogic smile. Long, dark hair cascaded down her back. A black T-shirt stretched tightly over her basketball of a belly. The scents of homemade cookies and comfort- food casseroles wafted up from the finished basement, where the crowd was settling in on chairs, cushions and earth-toned rugs.

Over the course of the next few hours, the women would kneel before her, reading excerpts from Kahlil Gibran’s “The Prophet,” telling her how much they loved and admired her and offering small, symbolic stones and charms that she would make into a necklace to wear during labor.

Dodie Sarnoff, Katie’s mother-in-law, approached with her contribution. “Here are your prayers. I don’t know if you’re going to look at them when you’re in labor, but. … “

Next came Katie’s mother, Jan Sauter, who had driven in from Michigan with Katie’s sister.

“A pearl from my mom’s pearl necklace and a charm that says ‘I love my family,’-” Sauter said, as both she and her daughter broke down in tears.

Later, Sauter said, “I didn’t think it would be this emotional.” A devoted Catholic, she said that “the pagan aspect was a bit of a shock.” To see how loved her daughter is by so many friends, she said, more than made up for the strangeness of this ceremony. “All you want is for your daughter to be happy.”

“Childbirth is the most profound change in a woman’s life, physically, emotionally and hormonally,” says Kathleen Furin, cofounder of the Maternal Wellness Center in Mount Airy, Pa. Furin, a social worker and mother of two, is one of the childbirth educators who organize these ceremonies.

An alternative party

Over the last seven years, Furin has arranged nearly a dozen birth circles. She has had two of her own before her two sons were born and is putting together one for her sister, due this month.

She prefers to call them “birth circles” because the term “blessing way” is sacred to the Navajo.

Instead of unwrapping gifts, the mother-to-be is surrounded by friends and relatives who may pamper her, clean her house, prepare food or write wishes on pieces of paper hoping for an easy labor or the baby’s health.

Depending on the woman’s degree of comfort with New Age rituals, the circle may also involve chanting, prayer and other ceremonial group activities.

All of this helps remind the woman that she is not alone, that giving birth is instinctive and as ancient as life itself, and that throughout her labor, her friends and family will be thinking of her.

“Baby showers are centered around the baby,” explains Joan-E Rapine, a childbirth educator from Medford,, who also has organized circles for several friends. “At a blessing way, you’re focusing on the mother, bringing her blessings and love. For that evening, she’s the queen. She’s the center of attention.”

In Western society, most of the preparation for birth is physical and economic, Furin says. Women take vitamins and get prenatal checkups. They prepare nursery rooms and plan for medical bills and childcare. “There is a tendency not to pay attention to the emotional side of birth.”

Furin says that almost any woman in her ninth month of pregnancy can benefit from a few hours of doting encouragement. A key part of the ceremony is to allow the mother-to-be to express her hopes and fears.

While many of the women who opt for birth circles are also inclined to choose natural childbirth, “there is no one right way to give birth,” Furin says. “What matters is that the woman feels somewhat in control, that she feels safe and well cared for.”

At Furin’s first birth circle, her friends told her to go take a shower while they cleaned her house and decorated it with a profusion of flowers. When she came downstairs, they massaged her feet and wove flowers into her hair. They also painted her belly, put small shells and stones into a pouch to represent their wishes for her, and tied pieces of string around their wrists. When she went into labor about a month later, each friend lit a candle, she said. “I knew my friends were thinking of me.” After her son was born, as each friend got the news, they cut off the string.

“It’s about empowering women through labor,” says Beth Ann Corr, an acupuncturist in Mount Airy who had a circle ceremony in December before the birth of her daughter. “It gives you a sense of community, reminding you that millions of women have gone through this before you, and you can do it, too.”

Corr, who is in her 30s, says she found the ceremony to have a lasting and powerful effect that helped her stay focused for much of her labor.

She chose to have a plaster cast made of her pregnant belly, which her friends decorated.

“I used to sculpt a lot. It was neat to have this remembrance of my first baby. I keep it in the nursery. It’s decorated with dried flowers and is actually quite beautiful.”

Sarnoff had a belly cast made in advance of the blessing way. It sat on a table throughout the ceremony, chalky white and ghostly but elegant still, in all its armless-Venus glory. Her friends wove a wreath of flowers and placed it on her head. Her husband, Michael, sat beside her, massaging her lower back.

As the only man in the room, he felt no discomfort, only pride. “If it weren’t for me, none of them would be here!”

Helping mom’s psyche

The ceremonies, which are more common in California, Australia and Canada, are not well known in this region. “When I send out an invitation, the friends of the mother usually don’t know what it is,” Rapine says. “So I tell them you could call it a ‘spiritual baby shower.’-“

Although there are some core aspects to the ritual, she cautions that each ceremony must be planned according to the woman’s wishes. It is also important to consider whether the people invited will be open to participating.

“Some people might think it’s a little goofy,” says Corr. “But when you look at what you’re doing, it’s to support the person. Going into labor is scary the first time, and it’s helpful knowing you can express that fear freely if you want to.”

“I wasn’t sure if it was something I wanted,” says Melissa Rooney, a 34-year-old social worker from Mount Airy. “I’m not particularly religious or spiritual, but the more I heard about it, the more I thought I could benefit from the emotional support.”

Rooney had heard about the ceremony from Furin.

“I have three sisters,” Rooney says. “I thought they would laugh, but Kathy said that was OK. We didn’t do a whole lot of ritualistic stuff. I read a poem that put more eloquently than I could what I was hoping for. … Everyone offered wishes for my birth and my expanding family. They decorated my belly, massaged my feet and shoulders, then we ate.”

She giggled during the rituals, she says. “Not in a this-is- ridiculous way, but more like this is an interesting experience. I wasn’t thinking anything would be transformed.”

One of her sisters made her a pouch, and everyone wrote down a wish or brought a token to symbolize a wish. “My plan was to have that with me during labor,” Rooney said.

Before she went into labor, a problem occurred, and she needed a C-section.

“I think what the birth circle did do for me was to help me feel ready for whatever came and know that you can’t always control these things.”

At the end of Sarnoff’s ceremony, as her guests roamed the room with paper plates full of comfort food, she said, still a little teary, “It was beautiful.”

Dyspnea, Hemoptysis, and Perihilar Infiltrates in a 35-Year-Old Man*

(CHEST 2005; 127:1437-1441)

A 35-year-old man was admitted to the hospital with the chief complaint of progressive dyspnea on exertion for 3 weeks. He also reported a history of night sweats and weight loss of 2 kg within this time period. He had a cough productive of white, occasionally blood-streaked sputum.

Physical Examination

On hospital admission, the patient’s temperature was 37.1C, heart rate was 97 beats/min and regular, BP was 109/67 mm Hg, respiratory rate was normal, and the transcutaneous oxygen saturation was 95% on room air. The cardiac and pulmonary examinations were normal. There was no jugular venous distention, lymphadenopathy, cyanosis, clubbing, or edema. The abdomen was soft without evidence of hepatosplenomegaly.

Laboratory and Radiographic Findings

A CBC count revealed normocytic anemia (hemoglobin, 11.4 g/L; mean corpuscular volume, 83 femtoliters) and a leukocytosis of 14.3 10^sup 9^/L. Results of serum chemistries, urinalysis, and coagulation were normal. Arterial blood gas analysis on room air showed a mild respiratory alkalosis and an elevated alveolar- arterial O2 difference (pH 7.46; PCO^sub 2^, 33.3 mm Hg; PO^sub 2^, 53 mm Hg; HCO^sub 3^, 24.1 mEq/L; and oxygen saturation, 89.3%). ECG showed sinus tachycardia at a rate of 115. Chest radiography (Fig 1) revealed increased opacity throughout both lung fields, which was thought secondary to underpenetration. Three sets of sputum were negative for acid-fast bacilli. Blood culture findings were negative, CD4 count was normal, and an HIV test was not sent. Serum antinuclear antibody, antineutrophil cytoplasmic antibody (ANCA), anti-glomerular basement membrane antibody (anti-GBM), coccidioidomyces, and cryptococcus titers were all negative. Serum C3 and C4 levels were within normal limits.

FIGURE 1. Posteroauterior chest radiograph obtained on the day of admission. There is increased opacity throughout both lung fields.

A ventilation/perfusion scan of the lungs was low likelihood for pulmonary embolism. CT of the chest (Fig 2) revealed extensive ground-glass opacities with predilection of the perihilar lung areas bilaterally; no lymphadenopathy was noted. Echocardiogram showed trace tricuspid regurgitation without other evidence of valvular abnormality. Pulmonary function tests revealed the following: FEV^sub 1^, 2.39 L (predicted, 3.5 L; 68% of predicted); FEV^sub 1^/ FVC, 81% (predicted, 84%); total lung capacity, 3.49 L (predicted, 5.91 L); and diffusing capacity of the lung for carbon monoxide, 32.2 mL/min/mm Hg (predicted, 25.9 mL/min/mm Hg).

Bronchoscopy was performed. The lavage specimen was grossly bloody, and numerous hemosiderinladen macrophages were seen on cytology. The patient then underwent video-assisted thoracoscopic biopsy, with the specimen showing pulmonary hemorrhage with hemosiderosis. Immunofluorescence studies (Fig 3) were positive for linear staining of IgG on the alveolar basement membrane.

FIGURE 2. Chest CT scan showing extensive ground-glass opacities predilecting the perihilar regions.

FIGURE 3. Immunofluorescence of lung biopsy tissue showing linear deposition of IgC on the alveolar basement membrane (original 60).

What is the likely diagnosis, and what treatment should be instituted?

Answers: Goodpasture syndrome with negative serum anti-GBM antibody and normal renal function. Treatment should consist of corticosteroids, cyclophosphamide, and plasmapheresis.

This patient presented with clinical and radiographic features of diffuse alveolar hemorrhage, an entity with a broad differential diagnosis and a potentially catastrophic outcome, with an acute mortality of up to 50%. Prompt diagnosis should therefore be pursued concurrently with stabilization of the patient. In this patient, drug use (ie, crack cocaine inhalation, propylthiouracil, diphenylhydantoin, penicillamine, amiodarone, nitrofurantoin) and environmental exposures (ie, hydrocarbons, infection) were excluded. Given the paucity of systemic findings, the differential diagnosis included anti-glomerular basement membrane disease (Goodpasture syndrome) confined to the lung, isolated pulmonary capillaritis with or without autoantibodies, antineutrophil cytoplasmic antibody- associated vasculitides (Wegener granulomatosis with isolated pulmonary involvement, or microscopic polyangiitis), Henoch- Schnlein purpura, and cryoglobulinemia. Less likely syndromes in this case included systemic lupus erythematosus, collagen vascular diseases such as systemic sclerosis, dermatomyositis, and antiphospholipid syndrome. Sending serum for ANCA, anti-nuclear antibodies, and anti-GBM antibodies, as well as obtaining a careful history and routine electrolyte, renal function, hematology and coagulation studies, were therefore critical in narrowing the differential diagnosis.

Goodpasture syndrome is a rare autoimmune disease, with an incidence of approximately 0.1 cases per million, which is characterized by pulmonary hemorrhage, crescentic glomeruonephritis, and the presence of circulating anti-GBM antibodies. The antibodies are directed against the noncollagenous domain of the α-3 chain of type IV collagen (α-3[IV]NC1), which is the major type of collagen in glomerular and alveolar basement membranes. There is a bimodal age distribution, with peaks of incidence in the fourth and seventh decades of life. Exposure to hydrocarbons in industrial settings has been proposed as a risk factor. Genetic associations with the major histocompatibility complex class II alleles human leukocyte antigen-DR2 and human leukocyte antigen-DR15 have been reported. Multiple theories about the possible pathogenic mechanisms exist; it is currently thought that an inciting process in susceptible individuals initially damages the GBM and exposes the α3(IV)NC1 epitope, thereby stimulating antibody production and subsequent inflammatory responses.

Hemoptysis is the most common presenting symptom in Goodpasture syndrome, occurring in 65% of patients in one series. Other common symptoms include progressive dyspnea, cough, and fatigue. Fewer than 10% of patients present with gross hematuria, although the incidence of microscopic hematuria on urinalysis is much higher. Physical examination may be notable for pallor and tachycardia secondary to anemia and diffuse crackles on lung auscultation. Routine laboratory studies commonly reveal anemia from chronic blood loss, as well as varying degrees of renal insufficiency. Chest radiography may reveal patchy or diffuse airspace consolidation with air bronchograms, corresponding to areas of hemorrhage. These areas tend to occur in the perihilar region and in the middle and lower lung zones; the apices and costophrenic angles are usually spared. The chest radiograph may be normal in about 20 to 25% of cases. CT may reveal areas of ground-glass attenuation or consolidation with a predilection for dependent lung zones.

The “gold standard” for diagnosing Goodpasture syndrome is the finding of a linear pattern of immunofluorescence on the glomerular or alveolar basement membrane; this requires a lung or kidney biopsy. In the setting of a classic presentation of diffuse alveolar hemorrhage and renal failure, however, a positive serum anti-GBM antibody can establish the diagnosis and preclude the need for biopsy. Some experts still advocate performing a renal biopsy in all suspected cases, because the degree of crescentic glomeruonephritis observed and the number of glomeruli involved can affect the aggressiveness of therapy. Diabetic nephropathy and fibrillary glomerulonephritis are two other conditions characterized by linear deposition of IgG, but in these conditions IgG is nonspecifically absorbed onto the highly permeable capillary wall and onto the fibrils respectively. Moreover, in diabetic nephropathy there is linear deposition of albumin and other plasma proteins. The serum ANCA can be positive in 10 to 38% of patients with positive anti- GBM; their antigen specificities are the same as in patients with anti-GBM alone, but these patients appear to often have more treatable disease.

The prognosis of Goodpasture syndrome was uniformly grim before the development of plasma exchange and powerful immunosuppressive drugs. Treatment consists of oral prednisone, starting at 1 mg/kg/d with a gradual taper, and oral cyclophosphamide, 2.5 mg/kg/d. Daily plasmapheresis should be administered for a total of 14 sessions or until the serum anti-GBM titer is undetectable. Patients should refrain from smoking, and any intercurrent infections should be treated aggressively as they can precipitate relapse. The long-term outcome with treatment depends on the degree of renal involvement. Patients with normal renal function at diagnosis appear to have the best prognosis, although they may still have relapse of pulmonary symptoms. In contrast, many patients who initially present with renal failure will eventually need dialysis despite treatment.

Unusual Features of this Patient’s Presentation

Several features of this patient’s presentation deserve comment. He was a nonsmoker, and smokers have accounted for 72 to 80% of cases in recent series. Smoking is also associated with a higher risk of pulmonary hemorrhage, as opposed to renal manifestations alone, in patients with anti-GBM disease. This patient also presented with normal renal function and no gross or mi\croscopic hematuria. Urinalysis was normal in 10% of patients in one case series, and several cases of Goodpasture syndrome with normal renal function have been reported. Even in patients who acquire both pulmonary and renal disease, the pulmonary symptoms tend to precede the kidney findings by months to years.

This patient also presented with a negative serum anti-GBM antibody by enzyme-linked immunosorbent assay. Up to 40% of patients with Goodpasture syndrome have a false-negative immunofluorescent antibody test result; radioimmunoassay or enzyme-linked immunosorbent assay using native and recombinant α-3(IV)NC1 is more sensitive, but the results take longer to return. In a few reported cases, IgA was the main circulating antibody, and was therefore not detected by commercial assays, most of which only test for IgG. Patients with a negative circulating anti-GBM antibody appear to have more benign kidney disease, with lower rates of progression to hemodialysis; the reasons for this are still unclear. Western blot, performed mostly in reference laboratories, is a more sensitive and specific method for detecting circulating anti-GBM, and may be considered if the diagnosis is in doubt; it is usually not necessary unless the patient refuses or has contraindications to biopsy. The development and widespread use of biosensor systems, which are even more sensitive than Western blot, may substantially decrease the rate of seronegative Goodpasture syndrome in the future.

This patient also underwent lung biopsy, rather than renal biopsy, in order to make the definitive diagnosis of Goodpasture syndrome. The sensitivity and specificity of the two approaches have never been compared in a formal trial. The clinical context will determine which technique is used, although in most cases renal biopsy is safer and more convenient for the patient, and it allows an assessment of the degree of glomerular damage.

Follow-up

The patient was discharged without a formal diagnosis. He was started on prednisone, 60 mg/d po, with marked resolution of his symptoms. His biopsy result was consistent with Goodpasture syndrome, and he was readmitted a week after discharge for plasmapheresis. He received nine courses of plasmapheresis, and was also started on cyclophosphamide. The patient continues to feel well with no recurrence of his pulmonary symptoms.

CLINICAL PEARLS

1. In a patient presenting with progressive dyspnea, hemoptysis, radiographic patchy opacities sparing the apices, ground-glass infiltrates on chest CT, and an increased diffusion capacity, diffuse alveolar hemorrhage should be high in the differential diagnosis.

2. Although Goodpasture syndrome is a rare cause of diffuse alveolar hemorrhage, it should be excluded, especially in smokers or those who present with renal insufficiency or hematuria.

3. If there is a high clinical suspicion for Goodpasture syndrome, a negative serum anti-GBM antibody is insufficient to exclude the diagnosis and does not eliminate the need for biopsy.

4. Patients with Goodpasture syndrome who have normal renal function and/or no hematuria on initial presentation appear to have a more benign course.

5. Prompt diagnosis of Goodpasture syndrome is essential, as early therapy with corticosteroids, cyclophosphamide, and plasmapheresis can favorably alter the prognosis.

ACKNOWLEDGMENT: The authors thank Dr. Anton Mlikotic for providing the radiographic images. Dr. Samuel French furnished the pathology specimen. Dr. Harry Prince of Focus Laboratories and Dr. Holly Mason gave insight into the basis of the anti-GBM enzyme- linked immunosorbent assay.

* From Harbor-UCLA Medical Center, Torrance, CA.

SUGGESTED READINGS

Alpers CE, Rennke HG, Hopper J, et al. Fibrillary glomerulonephritis: an entity with unusual immunofluorescence features. Kidney Int 1987; 31:781-789

Bolton WK. Goodpasture’s syndrome. Kidney Int 1996; 50:1753-1766

Burns AP, Fisher M, Li P, et al. Molecular analysis of HLA class II genes in Goodpasture’s disease. QJM 1995; 88:93-100

Carre P, Lloveras JJ, Didier A, et al. Goodpasture’s syndrome with normal renal function. Eur Respir J 1989; 2:911-915

Fraser RS, Mller NL, Colman N, et al, eds. Fraser and Par’s diagnosis of diseases of the chest. 4th ed. Philadelphia, PA: W.B. Saunders, 1999; 1757-1769

Green RJ, Ruoss SJ, Kraft SA, et al. Pulmonary capillaritis and alveolar hemorrhage: update on diagnosis and management. Chest 1996; 110:1305-1316

Hellmark T, Niles JL, Collins AB, et al. Comparison of anti-GBM antibodies in sera with or without ANCA. J Am Soc Nephrol 1997; 8:376-385

Kalluri R, Myers K, Mogyorosi A, et al. Goodpasture syndrome involving overlap with Wegener’s granulomatosis and antiglomerular basement membrane disease. J Am Soc Nephrol 1997; 8:1795-1800

Knoll G, Rabin E, Burns BF. Antiglomerular basement membrane antibody-mediated nephritis with normal pulmonary and renal function: a case report and review of the literature. Am J Nephrol 1993; 13:494-496

Lamriben L, Kourilsky O, Mougenout B, et al. Goodpasture’s syndrome with asymptomatic renal involvement: disappearance of antiglomerular basement membrane antibodies deposits after treatment. Nephrol Dial Transplant 1993; 8:1267-1269

Salama AD, Dougan T, Levy JB, et al. Goodpasture’s disease in the absence of circulating anti-glomerular basement membrane antibodies as detected by standard techniques. Am J Kidney Dis 2002; 36:1162- 1167

Salama AD, Levy JB, Lightstone L, et al. Goodpasture’s disease. Lancet 2001; 358:917-920

Shah MK, Hugghins SY. Characteristics and outcomes of patients with Goodpasture’s syndrome. South Med J 2002; 95:1411-1418

Westberg NG, Michael AF. Immunohistopathology of diabetic glomerulosclerosis. Diabetes 1972; 21:163-174

Chin Jung, MD; George Karpouzas, MD; and William W. Stringer, MD, FCCP

Manuscript received January 8, 2004; revision accepted March 31, 2004.

Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (e-mail: [email protected]).

Correspondence to: Chin Jung, MD, Department of Medicine, Box 400, LA. County Harbor-UCLA Medical Center, Torrance, CA 90509; e- mail: [email protected]

Copyright American College of Chest Physicians Apr 2005

Laura Ingraham’s Breast Cancer Highlights Need for Early Detection

NORTHFIELD, Ill., April 26 /PRNewswire/ — Breast cancer is the most common cancer among women and the second leading cause of cancer deaths. Conservative radio talk show host and best selling author Laura Ingraham’s recent encounter with this potentially deadly disease illustrates the importance of early detection in diagnosis and treatment.

“Timing is critical when it comes to cancer — the sooner it is detected the better the chance a woman has to get successful treatment,” said Sue Hilton, MD, FCAP, at St. Paul Medical Center in Dallas, Texas. “Even though women know that early breast cancer detection may save their life, many say they are ‘too busy’ or simply forget to schedule their annual mammograms.”

Mammograms can help detect cancer before a woman can feel a lump in a self-examination, as well as detect cancers too small to feel during a clinical breast exam.

Dr. Hilton, a physician who specializes in treating patients through laboratory medicine, says all women should monitor their breast health on a regular basis. The College of American Pathologists recommends that all women 40 years and older should have annual mammograms, based on their physician’s recommendation.

“Breast cancer can strike women at any age,” Dr. Hilton said. “It is especially important to start regular screening early if you have a family history of breast cancer.”

Other risk factors for breast cancer are:

— Personal history of breast cancer: women with cancer in one breast have

a higher chance of developing a new cancer (not just a recurrence of

the earlier cancer) in another part of the same breast or in the other

breast.

— Race: While Caucasian women are slightly more likely to develop breast

cancer than African-American women, African-American women are more

likely to die of breast cancer. Asian and Hispanic women have a lower

risk of developing breast cancer.

— History of breast biopsy: Certain types of abnormal breast biopsy

results can be linked to a slightly higher risk for breast cancer.

— History of radiation treatment: Women who had chest area radiation

treatment as children or young women have a significantly increased

risk for breast cancer.

In response to surprising data from a 2000 Gallup poll of 1,000 women, the College of American Pathologists created a free health test reminder Web site. According to this national poll, 36 percent of all adult women did not have a Pap test in the prior 12 months. Yet the poll results indicated that women who receive a reminder to schedule a Pap test are more likely to report getting a Pap test within the past 12 months than women who do not receive a reminder (78 percent vs. 47 percent, respectively). Among those surveyed who said that they did not receive a reminder, 62 percent felt that they would be more likely to make an appointment if reminded.

“It was surprising that a simple reminder had such a huge impact on the health of so many people,” said Dr. Hilton. “It became obvious that something had to be done to help busy patients remember to schedule their potentially life-saving tests.”

That “something” was a new reminder Web site. In less than one minute, a person can log onto http://www.myhealthtestreminder.com/ , register and choose the health test reminder(s) they want to receive — mammogram, Pap test or colon cancer screening — and when they would like to receive the reminder(s). The site automatically will send a private e-mail reminder on the requested date.

The College of American Pathologists is a medical society serving nearly 16,000 physician members and the laboratory community throughout the world. It is the world’s largest association composed exclusively of pathologists and is widely recognized as the leader in laboratory quality assurance. The CAP is an advocate for high quality and cost-effective patient care.

Take One Minute to Beat Cancer

1) Visit http://www.myhealthtestreminder.com/ .

2) Choose which screening reminder(s)– Pap test, mammogram or colon

cancer screening — you would like to receive and when you would like

to receive it.

3) Log off the computer and get back to your busy life.

4) As soon as you receive your e-mail reminder, call your doctor and

schedule your cancer screen.

College of American Pathologists

CONTACT: Patti Flesher, or Diane Simpson, +1-800-323-4040, ext. 7538, orAnthony Phipps, +1-800-323-4040, ext. 7574, all for College of AmericanPathologists, [email protected]

Web site: http://www.cap.org/http://www.myhealthtestreminder.com/

Good Question: Worry About Aluminum in Antacids, Not in Aspirin

Question: I have read that enteric-coated aspirin contains aluminum. Can you tell me if this is true? Can you tell me what “enteric coating” is made of? And can you tell me what evidence there is that ingesting aluminum regularly may cause health problems? This seems to me to be especially important now that many people are taking baby aspirin daily on the advice of their cardiologists. – Aku, also known as Ed Oppenheimer, over 50 years old, Santa Fe

Answer: Navigating the dos and don’ts of aluminum consumption can be downright confusing.

Aku, a textile artist, has been hesitant to follow his cardiologist’s suggestion to take aspirin regularly because of unresolved concerns about the presence of aluminum in aspirin.

“It’s possible it’s another big mistake by the medical profession,” Aku said, of the common advice to take aspirin.

He refuses to take it. Instead, he uses ginkgo, an herb with blood-thinning qualities.

“It seems to do the job as well as aspirin,” he said.

In large quantities, aluminum can be toxic, especially for people with kidney problems.

Michael Lacey, director of pharmacy at St. Vincent Regional Medical Center, remembers taking a set of aluminum pots and pans back to the store because he was concerned that eating food cooked in them would dump too much aluminum into his bloodstream. “The fear is dementia and Alzheimer’s can be caused by aluminum,” he said.

But when it comes to aspirin, he’s not worried. To him, a more serious concern lies in antacids with aluminum, which carry a far bigger dose than aspirin.

“The risk of aluminum toxicity resulting from the enteric coating in a person without kidney disease is zero,” Lacey said. “The benefit of taking aspirin in a male over 50 years old has been shown repeatedly to reduce heart attack and stroke in this population. There is no question that, from a health perspective, it is in this person’s best interest to take the aspirin.”

Lacey explained the ins and outs of enteric-coated aspirin.

Aspirin is known to cause stomach bleeding. Enteric coatings, some of which contain aluminum hydroxide, are applied to tablets to allow aspirin to bypass the stomach and dissolve in the small intestine, he said. The coatings are made of starch-like chemicals that come from wood. Aluminum hydroxide is sometimes used as a component of the dye for the coating. In enteric-coated aspirin, it’s known as FD&C #6 Aluminum Lakes, he said.

Aluminum is everywhere. It is one of the most plentiful elements in nature. Drinking water, fruits and vegetables, fish, meats, cooking utensils, cosmetics and even cheeseburgers add to the daily dose. The average person consumes 2 to 8 mg per day. Automatically, the body flushes most of it out.

“When aluminum is present as an ingredient in an enteric-coating formulation,” Lacey said, “it would be in extremely small amounts, similar to aluminum content in the diet. I don’t think you’d get one- tenth of one milligram from the coating on aspirin.”

By comparison, some antacids contain up to 100 mg of aluminum per dose, he emphasized.

For a healthy person, large amounts of the metal are the problem. For a pregnant woman or a person with poor-functioning kidneys, smaller doses can have a stronger effect.

“There is evidence that ingestion of large quantities of aluminum can cause toxicity in humans, particularly when the quantity ingested exceeds the body’s capacity to eliminate it,” Lacey said.

For a deeper discussion, he recommended emedicine.com on the Internet.

Aluminum toxicity was originally described in the mid-to-late 1970s in a series of patients in Newcastle, England, and remains a rare condition, according to the site. Excess aluminum is deposited in various tissues, including bone, brain, liver, heart, spleen and muscle.

Since the role of aluminum in disease has been identified, more attention has been paid to the element. Aluminum causes an oxidative stress within brain tissue, leading to the formation of Alzheimer- like neurofibrillary tangles. Excess aluminum has been shown to induce anemia. Children might have varying degrees of growth retardation.

Recent case reports have implicated the use of aluminum- containing antacids during pregnancy as a possible cause for abnormal fetal neurologic development.

The signs and symptoms of aluminum toxicity may include muscle weakness, bone pain, multiple non-healing fractures, acute or subacute alteration in mental status and premature osteoporosis. These patients almost always have some degree of renal disease.

According to the Web site, medical staff should educate pregnant and breastfeeding females, and any patient with compromised kidney function, about the potential dangers of the use and overuse of aluminum-containing antacids. A safe alternative includes calcium carbonate, such as that found in Tums.

E-mail health and science questions to: [email protected] or mail them to Good Question, The Santa Fe New Mexican, 202 E. Marcy St., Santa Fe, NM 87501. Please include your name, telephone number, age and city.

WHY DO I KEEP GETTING ACNE? Britain’s Top Integrated Health Expert

Q I am 16 years old and suffer from acne. I have horrible scars from previous spots, which make me very self-conscious. I recently heard of a contraceptive pill called Dianette that helps to prevent acne. Would you recommend it or do you have any alternative treatments?

A This inflammatory skin disease, which affects the skin pores and the oil glands attached to them, is an abnormal response to normal levels of the male hormone testosterone in the blood. Both male and female reproductive hormones are made from cholesterol. The male hormones (androgens), including testosterone, are synthesised first. Then, in women, they are converted to oestrogen in the ovaries. Additionally, androgens in the adrenal glands are converted to oestrogen in fat cells.

During puberty, however, the upsurge of hormone production reaches such a pitch that many boys have an excess of testosterone. Girls also have a surplus because the ovaries can’t convert it all to oestrogen. The body tries to expel this surplus through sebaceous glands in the skin.

These glands produce oil (sebum) to keep skin and hair waterproof.

Generally, all fat-soluble excess substances are excreted through sebaceous glands. As hormones are fat-chain complexes, they are deposited in the sebaceous glands and produce the characteristic yellow pus. Germs, given the opportunity to enter the sebaceous glands (eg, when a spot is touched), have a feast. This causes inflammation. The infected pus builds up and, when ripe, the spot bursts open, releasing the contents on to the skin. The cavity then fills with scar tissue, which ultimately causes pigmentation. If you squeeze spots prematurely, you spread the infection by pushing the pus over surrounding skin. The result is a larger area of scarring.

In principle, acne is the body’s cleaning up process so it’s a useful function. Unfortunately, it is unsightly. The clever thing to do is to work with the body.

My advice is to keep the skin hygienic and healthy so that the spots don’t get infected.

I also believe that if the body’s general functions improve, hormones will become more balanced.

Dianette (also a contraceptive pill) blocks the process whereby the androgens are excreted through the skin.

It can work for acne although it may take several months. Dianette is an anti-androgenic progestogen, which blocks the receptors in the body that the androgens work on. (Progestogens are a group of female reproductive hormones.) As with all prescription drugs, talk to your doctor about all the risks and side effects before deciding whether or not to take it.

Many people think that acne is caused by poor diet. This is a myth, but eating fatty and sugary foods and drinking orange juice, which encourages inflammation of the skin, can weaken the body’s ability to function well. A good diet helps you to cope with any disease better.

. Help your digestion by chewing food well.

. Make yourself fresh fruit and vegetable juices daily: try combining carrot, celery, apple and ginger.

. Eat oily fish (for essential fatty acids), avocados (for vitamin E), carrots (for betacarotene), pomegranates (to fortify blood), and yams (to help hormonal balance).

. Add one dessertspoon of wheatgerm daily to your food.

. Avoid yeast products, deep fried foods, chocolates, sweets, excess salt, alcohol, coffee and citrus juice.

. Drink two litres of still water daily between meals.

. Zinc (Biocare zinc citrate, Pounds 4.50 for 90 tablets): take one daily for two months for skin maintenance.

. Kadu and kariatu: soak three kadu twigs and one third of a teaspoon of kariatu in a cup of hot water at night; in the morning, strain, add a little hot water then drink. This acts as a good detox. (Kadu, Pounds 4 for 50g; kariatu, Pounds 4.50 for These are my suggestions Diet . Choose organic produce.

Supplements 100g, both Top Op Foods.) . Skin Oil (Alive, Pounds 6 for 20ml): apply on a cotton bud to sore spots in the evening, two to three hours before washing, to aid healing.

. Borolene cream (GD Pharma, Pounds 4.50 for 21g): massage this antiseptic cream into affected areas at bedtime.

To help scarring . Ganpati cream (Pounds 8): apply a small amount to the scar tissue and pigmented area after washing with mild soap and water. Leave overnight and wash off in the morning. Only use this on existing pigmented scar tissue, for two months.

. Apply pure aloe vera gel. Crush the leaves of an aloe vera plant (available from some health food shops), then apply the gel that oozes out with a cotton bud every morning. Also take two aloe vera capsules (Vega, Pounds 7.50 for 60) twice daily for three to four months.

. You could also try vitamin E oil. Prick a vitamin E capsule with a pin and apply the contents on to the scars. Rosehip oil may also help.

Complementary therapies I suggest that you consult qualified practitioners of homoeopathy and nutritional medicine, and use organic beauty products such as Dr Hauschka.

The Benefits of Infant Massage: a Critical Review

EVIDENCE BASED PRACTICE

Abstract

Baby massage classes are increasingly popular and many health visitors are undertaking training to run them. This paper looks at the evidence base for baby massage. A systematic review of the research-based evidence identified four articles that met the inclusion criteria i.e. they were research-based articles looking at the effects of hands-on massage of babies and infants. The findings are synthesised and the research is critically reviewed. Insufficient evidence is found for the continued massage of pre- term and low birthweight infants in neonatal intensive care units. Benefits are claimed for the use of massage in specific medical conditions such as asthma and dermatitis, but the review reporting on these benefits was methodologically flawed. A randomised controlled trial found no significant difference between colicky babies that were massaged and a control group. By far the most compelling evidence relates to the benefits of baby massage on the maternal-child relationship and postnatal depression. This evidence would thoroughly justify the provision of infant massage classes in the community by the health visitor.

Key words: Massage, infancy, childhood, health visitors, evidence- based practice, maternal-child relationship

Community practitioner 2005; 78, 3: 98-102

Rationale

Infant massage is one area of health visiting that always attracts enthusiastic interest and participation from the public, an interest that seems to be primarily derived from media presentation and word-of-mouth recommendation. Given the current demand for evidence-based practice, it seemed timely to conduct a critical review of the current evidence base supporting the teaching of massage techniques to parents.

Methods

An electronic search was carried out on specialist databases (MEDLINE Plus, AMED, British Nursing Index, CINAHL, EMBASE, the Cochrane Database of Systematic Reviews, Psych Info, Clinical Evidence and NeLH) looking for articles that met the following inclusion criteria:

1. They considered the effects of baby massage (on either the parent or on the baby).

2. They were research-based.

3. They were published in peer-reviewed journals. (Many of the articles identified came from parenting journals or from alternative magazines which were not peer-reviewed and therefore had to be excluded).

4. They were published in the last 10 years, a period chosen because earlier research in the field focuses almost entirely on the effect of massage on the premature hospitalised baby.

The author also contacted a number of experts in the field – Suzanne Adamson, Vivette Glover, Sylvie Htu – and their comments are included where relevant.

Findings

Seventy-six references were gathered in total. As two of the studies identified were systematic reviews,1-2 the papers included in these reviews were not looked at separately.

Of the 76 papers identified, four met the inclusion criteria:

* A systematic review1 of articles on massage therapy and therapeutic touch in children.

* A systematic review2 of articles on massage of the pre-term or low birthweight infant from the Cochrane Database of Systematic Reviews.

* A recent study3 looking at infant massage in the treatment of colic.

* A randomised controlled trial4 to determine whether massage classes could reduce maternal depression and improve mother-infant interaction.

The findings of the four studies are summarised in Table 1.

As the Cochrane Review2 provided a comprehensive analysis of the benefits of massage for pre-term and low birthweight infants in the hospital setting, a field which is of only marginal interest to health visitors, it was not thought necessary to include subsequent studies of the same subject.

Critical review of the findings

Pre-term/low birthweight babies

The vast majority of research on baby massage relates to the pre- term and low birthweight baby. This may be because touch is considered particularly significant to a premature baby: as Vimala McClure describes,5 ‘the premature baby’s first contact with human touch may bring pain – needles, probes, tubes, rough handling, bright lights’. (p158) It may be because the cost implications of looking after such children make government funding easier to obtain, or it may be because research breeds research.

Both of the literature reviews1-2 looked at the research relating to the pre-term infant. As a Cochrane systematic review, Vickers’ article2 is a rigorous analysis which critically assesses randomisation, blinding, outcome assessment, sample size and heterogeneity.

Blinding means keeping group assignment (for example, to treatment or control) secret from the study participants or investigators or both. Blinding is used to protect against the possibility that knowledge of allocation may influence patient response.

Of the seven studies of pre-term babies identified by Ireland and Olson,1 only two (Field et al 1986(6) and Scafidi et al7] meet the high standards of Cochrane. Three are excluded from the Cochrane Review2 altogether:

* Solkoff and Matuszak 1975(8) (no details given as to method of treatment allocation)

Table 1: Summary of findings

* Rausch9 (historical control group)

* Morrow et al10 (did not meet specified selection criteria).

The remaining two articles (Solkoff 1969(11) and White and Labarba12) are included but are nonetheless criticised for their ‘unclear blinding’ of randomisation and ‘inadequate’ blinding of the intervention.2 (p34, 36)

Furthermore, the Cochrane Review2 included 21 studies not identified by Ireland and Olson.1 The authors state that they were keen to include as many trials as possible; they may therefore have trawled more widely, tracked more citations, included unpublished studies and corresponded with experts to elicit the maximum number of studies for inclusion. They also state that they included studies on infants who only just met criteria for low birthweights or prematurity.

It is perhaps not surprising that the two studies reach different conclusions on the benefits of baby massage. Ireland and Olson1 decide that ‘there is sufficient evidence to recommend the use of massage therapy in preterm neonates’. (p62)

The benefits identified include rapid weight gain, increased levels of activity, more rapid habituation and fewer days hospitalised. However, these claims are poorly justified in the text.

The authors list the limitations of the studies (no baseline measures, absent statistical procedures, small sample size and lack of control for confounding variables) but no attempt is made to discriminate between them. It is therefore very difficult to make any judgement on the validity of the various stated results.

Vickers2 looks much more critically and in much greater detail at the studies. The finding that pre-term babies gained more weight was found to be of low clinical significance with statistically significant heterogeneity between the trials.

The finding that hospital stay decreased in length was strongly influenced by one study with a small sample size.

Statistical heterogeneity is said to occur when the results of individual studies are to some extent incompatible with one another.

The results regarding improvement on the Brazleton scales for habituation, motor maturity, and range of state were also found to be methodologically flawed and of low statistical significance.

Vickers2 concedes that for medically stable infants, infant massage carries a low risk of adverse effects. However, his overall conclusion is that ‘there is insufficient evidence of effectiveness to warrant wider use of pre-term infant massage’, (p10) a conclusion which is in direct contradiction to the Ireland and Olson study and which, given the rigour of the review, must be seen to occupy a high position in the hierarchy of evidence related to the issue.

The sick child

A second area of research on the benefits of baby massage has been to look at children with specific health conditions. Ireland and Olson1 included in their review seven such studies, which they examined together as a body of work.

They reported consistent findings of lower anxiety, improved mood, and lower stress hormones (salivary cortisol), findings which may explain some of the specific benefits related to particular conditions such as:

* increased co-operation and sleep in emotionally disturbed children13

* improved relaxation in children with post-traumatic stress14

* reduced pain in children with juvenile rheumatoid arthritis15

* reduced distraction to noise, increased classroom attention and improved relating to teachers in children with autism16

* better clinical response in children with atopic dermatitis17

* increase in peak air flow in children with cystic fibrosis.18

It is noteworthy that all these studies were carried out at the same site, the Touch Research Institute at the University of Miami, directed by Tiffany Field, which is dedicated to studying the effects of massage. Although this means that the Institute is partisan, it will also be aware of the particular requirements of researching this area. It is encouraging that, in his critical review, Vickers2 did not find fault with the two studies6-7 produced by this Institute.

Ireland and Olson1 are critical of several findings in the studies relating to sick children, in particular that no reliability or validity data are provided for measuring mood, relaxation, emotional problems or pain. However, the main problem for the re\ader of the paper is the methodological vagueness of the review itself.

Ideally, a systematic review should synthesise the findings of different studies to reach reliable, accurate and consistent conclusions.19 In this case, despite being written by an assistant professor and published in a peer-reviewed journal, the review is of dubious quality and rigour and its conclusions are therefore questionable.

The colicky child

The study by Huhtala et al3 looked at the effect of infant massage on the colicky symptoms of 58 healthy infants of six weeks or less. The randomised controlled trial was meticulously conducted but found no significant difference between those babies that were massaged and the control group which used a crib vibrator.

The effectiveness was measured using a structured ‘cry diary’ and a parental interview. Both groups showed a similar decrease in colicky crying and the authors concluded that the results reflect more the natural course of early infant crying and colic than a specific effect of the interventions.

Postnatal depression and mother-infant interaction

The effect of baby massage on postnatally depressed mothers and their babies was the subject of one study20 which found that during massage babies appeared less stressed and showed decreased levels of crying and salivary cortisol. After massage their sleep improved and less time was spent in ‘active alert states’. Compared to controls they gained more weight and had improved temperament and lower salivary cortisol levels.

More recently, Onozawa et al4 conducted a randomised controlled trial which allocated 34 mothers who scored >12 on the Edinburgh Postnatal Depression Scale (EPDS)21 to an infant massage group and postnatal support group or to a support group only (control group).

Changes in maternal depression and maternal-infant interaction were compared using EPDS scores and a five-minute video recording of face-to-face play. The video recordings were assessed using the global ratings for mother-infant interactions at two months by Fiori- Cowley and Murray (Onozawa et al4) and a random selection of recordings was similarly rated by an experienced independent rater who was blind to the study.

The study found there was a greater improvement in depression scores in the massage group than in the control group and that mother-infant interaction improved significantly in the massage group. In every dimension measured the massage group showed an improvement whereas the control group remained the same. The authors conclude that attending an infant massage class substantially facilitated mother-infant interaction for women with postnatal depression.

The research was well conducted, with no difference in baseline depression scores or other demographic variables between the two groups. The outcome measures were clear and appropriate and the assessment was independently validated. The major problems with the study were the small size and the high dropout rate.

Eight-hundred-and-thirty women were screened initially using the EPDS. Of the 581 who completed the questionnaire 91 scored >12 on the EPDS. Of these, 59 agreed to take part, 34 started the class and nine dropped out.

The main reason for dropping out appeared to be the time of the class. Thus, only 25 women completed all the sessions, 12 in the massage group and 13 in the control group. Of these, two mothers in the massage group and one in the control group could not complete the video recordings because their infants were not settled. The researchers have recently received funding to repeat the study on a larger scale and to follow up mothers and babies for a year.

If the results of the initial study are confirmed, it would be of considerable clinical significance as mothers with postnatal depression are known to have impaired interactions with their infants22-23 and it is now well established that children of mothers who are depressed in their early months have more behavioural and cognitive problems later.24

Implications for practice

In his systematic review Vickers2 expresses his exasperation that ‘the nursing literature abounds in unsystematic reviews, nonrandomised and uncontrolled trials and even general discussion papers cited as evidence in support of massage’. It is unfortunate that one of the literature reviews considered in this study1 did not appear to tackle the quality of the trials or their scientific basis in any systematic way, thus diminishing its credibility.

However, the fact that a study is not randomised or is inadequately blinded does not invalidate it completely. In the hierarchy of evidence to support the teaching of baby massage, a systematic review such as Vickers,2 must take a prominent position, but other evidence should also be considered. Such evidence would include well-designed trials without randomisation (including some of the 72 studies excluded by Vickers2 for various reasons), the experience of parents as stated in informal evaluations and questionnaires, and the testimony of respected authorities in the field.

Furthermore, there is no suggestion that baby massage is ineffective, dangerous, expensive, or in any way damaging. As Vickers2 concedes, it is non-invasive, does not require specialist equipment and can be implemented without undue disruption to routine care procedures. If taught to and implemented by the parents, the cost implications are small.

On the basis of this review, the strongest evidence by far supports the use of baby massage to enhance the maternal-child relationship and reduce postnatal depression.

The evidence is compelling, the research basis is sound and the findings are supported by more than one study.

John Bowlby25 found two conditions to enhance the development of bonding: the ability of the caregiver to be sensitive in understanding and responsive to the infant’s cues and the amount and nature of the interactions between them. It is not surprising therefore if massage enhances bonding and attachment. Setting up infant massage classes in the community to enhance bonding could definitely be advocated on the basis of the evidence reviewed here and the study by Onozawa et al4 suggests that this will have a significant effect on postnatal depression.

With regard to the pre-term baby, the evidence-base for infant massage is weak, with the possible exception of length of stay outcomes. However, the cost implications of reducing the length of stay are of such significance that a randomised controlled trial of a high standard looking at the use of massage in a UK hospital may be justified.

On the basis of this review it would be difficult to report with any confidence on the benefits of massage in the management of particular medical conditions. The practitioner would be advised to view the conclusions of Ireland and Olson1 with a critical eye and refer back to the original research before making any changes to practice.

The benefits of baby massage on the normal healthy child should not be overlooked. Nearly 50 years ago Harlow26 demonstrated with infant monkeys that given the choice between an artificial mother made of wire offering liquid nourishment and a soft cuddly artificial mother with no nourishment the infant monkeys chose the latter, demonstrating that contact and comfort appear to be more important than nursing comfort. For a resume of the many animal studies demonstrating the power of touch, the interested reader is directed to Elaine Schneider’s fascinating article on the subject.27

Conversely, the evidence from orphanages at the beginning of the 20th century, or more recently in Romania, suggests that touch deprivation can result in profound neurological damage or even death in an otherwise healthy baby.28

The benefits of baby massage on the healthy baby is a little researched area, and one which would be particularly relevant to the work of the health visitor with its emphasis on health promotion rather than treatment of illness.

Conclusions

This systematic literature review found that a number of benefits have been claimed for infant massage. Main claims include:

* rapid weight gain in pre-term infants

* fewer days hospitalised for pre-term infants

* specific benefits in specific medical conditions

* improvement in postnatal depression scores

* improvement in mother-infant interaction.

There is no evidence that baby massage is dangerous or damaging in any way. The most compelling research relates to the maternal- child bond and it would thoroughly justify the setting up of classes teaching baby massage techniques to parents.

The benefits of baby massage on the healthy baby is a little researched area, and one which would be particularly relevant to the work of the health visitor with its emphasis on health promotion rather than treatment of illness

On the basis of this review, the strongest evidence by far supports the use of baby massage to enhance the maternal-child relationship and reduce postnatal depression. The evidence is compelling, the research basis is sound and the findings are supported by more than one study

References

1 Ireland M, Olson M. Massage therapy and therapeutic touch in children: state of the science. Alternative Therapies in Health and Medicine 2000; 6, 5: 54-63.

2 Vickers A, Ohlsson A, Lacy JB, Horsley A. Massage for promoting growth and development of pre-term and/or low birth-weight infants (Cochrane Review). The Cochrane Library, Issue 4, 2002, Oxford: Update software, 2002.

3 Huhtala V, Lehtonen L, Heinonen R, Korvenranta U. Infant massage compared with crib vibrator in the treatment of colicky infants. Pediatrics 2000; 105, 6.

4 Onozawa K, Glover V, Adams D, Modi N, Channi Kumar R. Infant massage improves mother-infant interaction for mothers with postnatal depression. Journal of Affective Disorders 2001; 63, 201 – 207.

5 McClure V. Infant massage: a handbook for loving parents. London: Souvenir Press, 2001.

6 Field T, Schanberg SM, Scafidi FA et a\l. Tactile/kinaesthetic stimulation effects on preterm neonates. Pediatrics 1986; 77, 654- 658.

7 Scafidi FA, Field TM, Schanberg SM et al. Massage stimulates growth in preterm infants: a replication. Infant behaviour and Development 1991; 13, 167-188.

8 Solkoff B, Matuszak D. Tactile stimulation and behavioural development among low birthweight infants. Child Psychiatry and Human Development 1975; 1, 33-37.

9 Rausch PB. Effects of tactile and kinaesthetic stimulation on preterm infants. Journal of Obstetric, Gynaecologic and Neonatal Nursing 1981; 10,34-37.

10 Morrow CJ, Field TM, Scafidi FA et al. Differential effects of massage and heelstick procedures on transcutaneous oxygen tension in preterm neonates. Infant behaviour and Development 1991; 14, 397- 414.

11 Solkoff N, Yaffe S, Weintraub D, Blase B. Effects of handling on the subsequent developments of premature infants. Developmental Psychology 1969; 1, 765-768.

12 White JL, Labarba RC. The effects of tactile and kinaesthetic stimulation on premature infants. Developmental Psychobiology 1976; 9, 569-577.

13 Field TM, Kilmer T, Hernandez-Reif M. Pre-school children’s sleep and wake behaviour; effects of massage therapy. Early Child Development and Care 1996; 120, 39-44.

14 Field TM, Seligman S, Scafidi FA. Alleviating posttraumatic stress in children following Hurricane Andrew. Journal of Applied Developmental Psychology 1996; 17, 37-50.

15 Field TM, Hernandez-Reif M, Seligman S. Juvenile rheumatoid arthritis: benefits from massage therapy. Journal of Pediatric Psychology 1997; 5, 607-617.

16 Field TM, Lasko D, Mundy P et al. Brief report: autistic children’s attentiveness and responsivity improve after touch therapy. Journal of Autism Developmental Disorder 1997; 27, 333- 338.

17 Schachner L, Field TM, Hernandez-Reif M. Atopic dermatitis symptoms decreased in children following massage therapy. Pediatric Dermatology 1998; 15, 390-395.

18 Hernandez-Reif M, Field TM, Krasnegor J. Children with cystic fibrosis benefit from massage therapy. Journal of Pediatric Psychology 1999; 24, 2: 175-181.

19 Cook D, Mulrow C, Haynes R. Systematic reviews: synthesis of best evidence for clinical decisions. Annals of Internal Medicine 1997; 126, 378.

20 Field TM, Grizzle N, Scafidi FA. Massage therapy for infants of depressed mother. Infant Behavioural Development 1996; 19, 107- 112.

21 Cox JL, Holden JM, Sagovsky R. The detection of postnatal depression: development of the Edinburgh Postnatal Depression Scale. British journal of Psychiatry 1987; 150, 782-786.

22 Murray L. The impact of postnatal depression and infant development. Journal of Child Psychology and Psychiatry 1992; 33, 543-61.

23 Murray L, Cooper PJ. The role of infant and maternal factors in postpartum depression, mother-infant interactions, and infant outcome. In: Murray L, Cooper PJ. Postpartum depression and child development. London: Guilford Press, 1997.

24 Murray L, Fiori-Cowley A, Hooper R, Cooper P. The impact of postnatal depression and associated adversity on early mother- infant interactions and later infant outcome. Child Development 1996; 67, 2512-2526.

25 Bowlby J. Attachment and loss. New York: Basic Books, 1969.

26 Harlow HF. The nature of love. American Psychologist 1958; 13, 673-685.

27 Schneider E. The power of touch: massage for infants. Infants and Young Children 1996; 8, 3: 40-55.

28 Carlson M. Understanding the mother’s touch. On the Brain 1997/ 8; 7, 1.

Caroline Zealey

Health visitor

South Hams and West Devon PCT

Copyright TG Scott & Son Ltd. Mar 2005

Abortion Foes Use Ultrasound ; Focus on the Family Budgets Millions for Pricey Equipment

Last fall, the Alternatives Pregnancy Center in Denver got a $36,000 piece of equipment that some say is the latest, and arguably most effective, tool in combating abortions.

Maureen Yockey, the executive director, says the ultrasound machine empowers women by helping them make informed decisions about their body.

“God made women to nurture and protect children,” she said. “Sometimes the picture from an ultrasound will give them the courage to choose to protect life.”

That’s what Focus on the Family is hoping will ultimately happen.

The Colorado Springs-based evangelical Christian group has budgeted $4.2 million this year to place ultrasound equipment in 150 pregnancy crisis centers. The long-term goal for Operation Ultrasound is to by 2010 place another 650 machines in centers that counsel women on alternatives to abortion, according to Julie Parton, director of the Pregnancy Resource Ministry for Focus on the Family.

Critics charge the program is an attempt by conservatives to manipulate women and push an agenda to eliminate legal abortions.

Consider this from Crystal Clinkenbeard, spokeswoman for Planned Parenthood of the Rocky Mountains, which offers abortion services:

“I didn’t see anything on their Web site that said they handed women a picture of the ultrasound without trying to fulfill their goal of dissuading women from having an abortion,” she said after looking at the Focus on the Family Internet site touting the ultrasound program.

Places like the Alternatives Pregnancy Center won’t stop a woman from leaving the center to have an abortion, but Yockey said they certainly don’t recommend locations where abortions are performed, either.

“It’s not like it’s a big secret where they are,” she said.

Ultrasound expensive

Ultrasound has been around for decades, with professor Ian Donald of Scotland developing its practical applications in the 1950s.

It is commonly used for viewing the embryo during pregnancies, but at between $150 and $500 per exam, it can be cost-prohibitive.

State Rep. David Schultheis, R-Colorado Springs, has tried three times to get legislation passed that would make ultrasounds mandatory for anyone considering an abortion while more than 12 weeks pregnant. The bill has died in committee each time.

“I think it shows there is very little difference between a baby on the outside and one on the inside,” bill co-sponsor Rep. Greg Brophy, R-Wray, said. “When people realize that, they are less likely to terminate the pregnancy.”

However, that wasn’t the case with Carly Febrey, a 27-year-old college student.

When she found out she was six weeks pregnant last summer, Febrey was sure she would have an abortion.

“We’re incredibly poor, and it was the wrong time in our lives to be raising the child,” she said. “It’s a personal tragedy.”

When she made an appointment at a local abortion clinic – there are nine listed in Colorado by the National Abortion Federation – she was given an ultrasound. Febrey was asked if she wanted to see it.

“I think the nurse was surprised when I said ‘yes,’ ” Febrey recalled. “I remember I could see something, but it wasn’t recognizable, and there wasn’t a pair of eyes looking back at me. I think that could be traumatic in a late-term abortion. This was essentially like seeing a coffee bean.”

Ultrasound technology is simply another skirmish in a war that only progresses – or regresses – in court battles, said Randall Lake, an associate professor of communications at the University of Southern California who has watched the abortion debate over the years.

While Focus on the Family is free to provide pregnancy crisis centers with ultrasound equipment, Lake said the battle line won’t move until lawmakers succeed in making ultrasounds mandatory.

“That would certainly be litigated,” Lake said.

Sides differ on effectiveness

As for the effectiveness of ultrasounds actually changing the minds of those considering abortions, the evidence is sketchy and fraught with caveats.

Focus on the Family statisticians say through counseling alone, 57 percent of women decide not to have an abortion. When the ultrasound image is added to the mix, that number jumps to 79 percent.

According to a survey published in Massachusetts News, a pregnancy crisis center in Boston claimed 77 percent of women chose not to have the abortion – up from around 50 percent who only received counseling without viewing an ultrasound image.

But most surveys are done through the very centers that are actively trying to stop women from having abortions, according to Vicki Saporta, president of the National Abortion Federation – a nonprofit group that represents more than 400 abortion centers around the country.

“I don’t think their statistics are valid or can be trusted,” Saporta said. “They can’t be put in a peer review journal. I think it’s propaganda.”

Saporta also said that most women who come to an abortion clinic have already made up their minds to have the procedure and that “an ultrasound image isn’t going to dissuade them” from following through with their choice.

“Women are intelligent,” Saporta said. “They know they’re pregnant, and the image they see – especially that early in the pregnancy – doesn’t look like that ultrasound image you’re seeing on the cover of Time magazine.” She was referring to the issue with a grainy black and white image of a fetus deep into the pregnancy.

But Lisa Vorhees said the ultrasound was “the single biggest influence” in her decision to not abort three years ago.

The 39-year-old said she had already planned to have an abortion – in fact had already made an appointment at a Planned Parenthood center in Denver – when a friend asked her to go to the Alternative Pregnancy Center.

An appointment was set up for her to get an ultrasound, and what Vorhees saw stunned her. She was pregnant with twins.

“All we could see was two little round sacks,” she said. “I was thinking before I saw it that I have this life in me, but was I willing to turn my life upside down for it? At first, I wasn’t. It was one of the biggest, hardest decisions I ever had to make in my life but, yes, the ultrasound was the biggest decider for me.”

Vorhees said she remains supportive of legal abortions.

“I believe everyone should have the right to make that choice,” Vorhees said.

According to the National Abortion Federation, 1.3 million women make the choice each year to abort. And Clinkenbeard said the six Planned Parenthood sites in Colorado did about 7,900 abortions in 2004. Clinkenbeard said each woman who gets an abortion receives an ultrasound beforehand, but the option to view the image is up to each woman.

She said most choose not to view it.

“It’s very few – less than a quarter,” she said. “And the ones that do as far as changing their mind? Very, very few.”

A Swollen Neck/Commentary

Ylva Bengtsson, MD

Commentary

Raja Mouallem, MD

The following Brief Report was written by a resident. A discussion by a member of the resident’s faculty follows. We invite any resident to submit such articles, together with commentary by a faculty member.

Patient Report

A 12-year-old boy presented to this pediatrie emergency department (PED) with tender swelling of his neck. The swelling had slowly developed over the previous 2 days. There was no history of bites, trauma to the neck, or exposure to kittens, and he denied toothache. He was diagnosed with cervical lymphadenitis and treatment with oral clindamycin was initiated. The following day, because he was febrile and the swelling had progressed to where he was unable to open his mouth, he returned to the PED. His oral intake had decreased, but he maintained a normal urine output. He denied shortness of breath and the review of systems was otherwise negative.

His previous medical history was remarkable only for recurrent herpes labialis. There was no history of dental caries or abscess. The family history was also unremarkable. He lives with his mother, and there are no pets. Immunizations were up to date, and he did not have any allergies. He was not taking any medicines except for clindamycin.

Physical examination revealed an alert boy in no acute distress but with significant swelling of his neck (Figure 1). There was no stridor. His temperature was 37.5C, heart rate, 102 beats/minute, respiratory rate 24 breaths/minute, and blood pressure was 132/79 mm Hg. Pulse oximetry was 100% on room air. A crusted lesion was noted on the left side of his lower lip. Neck swelling extended from the angle of the mandible to the middle of the neck (zone 2) and was indurated and warm but not fluctuant. The left side was somewhat more swollen than the right. He was unable to open his mouth more than minimally secondary to pain. His mouth could, however, be opened passively. The teeth were intact and there was no gingival swelling. The floor of the mouth was tender anteriorly and his voice was muffled, but he was not drooling. The tympanic membranes were pale and intact bilaterally. The neck was supple with full range of motion, and he did not have torticollis. He was tachycardie. The remainder of the physical examination findings was normal.

Initial laboratory investigation included an elevated WBC count of 19,100/mm^sup 3^ The differential showed 84% neutrophils, 11% lymphocytes, and 5% monocytes. A basic metabolic panel was within normal limits. Screen for Epstein Barr Virus (EBV-monospot) and a blood culture were obtained.

At this point the diagnosis of Ludwig angina was suspected. Owing to concern about the continued patency of his airway he was given intravenous dexamethasone and placed on continuous cardiorespiratory monitoring. ENT was consulted.

Computed tomography (CT) of the neck with contrast revealed a 1.3 0.9 cm hypodense area posterior to the symphysis of the mandible, likely representing a necrotic lymph node (Figure 2). There was diffuse density of the subcutaneous fat in the submental region consistent with cellulitis and there were additional prominent lymph nodes in the submental and submandibular regions interpreted as reactive lymph nodes. The airway was patent and there were no abnormal neck masses.

Figure 1. 12-year-old boy with marked swelling of the neck extending from the mandible to just above the clavicle.

Diagnosis: Cervical Lymphadenitis Presenting as Ludwig Angina

Hospital Course

He was admitted to the pediatrie intensive care unit for close monitoring of his airway and was continued on intravenous dexamethasone clindamycin. Over the next two days, the swelling improved and he did not require surgical drainage. The screen for EBV (monospot) was negative as was the blood culture. He was discharged home to finish a ten-day course of oral clindamycin.

ClinPediatr. 2005;44:271-274

Department of Pediatrics, Division of Emergency Medicine. LSU Health Sciences Center, New Orleans, Louisiana.

Reprint requests and correspondence to: Ylva Bengtsson, MD, Children’s Hospital, 200 Henry clay Avenue. New Orleans, LA 70118.

2005 Westminster Publications, Inc., 708 Glen Cove Avenue, Glen Head, NY 11545, U.S.A.

Commentary

Raja Mouallem, MD

Department of Pediatrics, Division of Emergency Medicine, LSU Health Sciences Center, New Orleans, Louisiana

Ludwig angina was first described in 1836 by Wilhelm Frederick von Ludwig and is a potentially serious condition in which there is a bilateral infection of the sublingual and submandibular area that can lead to airway obstruction. The infection begins in the floor of the mouth and induces a rapidly spreading gangrenous cellullitis. It is spread along the fascial planes, not propagated by the lymphatic system. The rapid spread causes edema of the neck and glottis and, thus, may cause airway obstruction.

Patients typically present with bilateral submandibular swelling, neck pain, fever and elevation of the tongue. Other common symptoms are toothache, trismus, dysphagia, and breathing difficulties.1 The submandibular swelling is characteristically firm and painful. There is usually tenderness of the floor of the mouth causing elevation of the tongue. If swelling is bilateral, the tongue is displaced upward and backward forcing the mouth open and occasionally interfering with breathing.2 The lymph nodes are unaffected.

The majority of cases are caused by dental infections, most commonly from the second or third mandibular molars. Other causes include peritonsillar and parapharyngeal abscesses, oral lacerations, mandibular fractures, submandibular sialadentitis,3 and intravenous drug injection into neck veins.4

Infection is usually polymicrobial with pathogens consisting of normal mouth flora such as Streptococcus viridans, Staphylococcus aureus, Staphylococcus epidermidis, and anaerobes, particularly Bacteroides.5

Plain radiographs, CT, and magnetic resonance imaging (MRI) of the neck and chest should be obtained to demonstrate the extent of the soft tissue swelling and intrathoracic extension.

Early administration of parenteral antibiotics, careful monitoring of the airway, and surgical drainage if there are any signs of fluctuance or abscess are the cornerstones of treatment.6,7 Initial antibiotic coverage is usually with penicillin, clindamycin, and metronidazole to cover for grampositive cocci and anaerobes.7 Some experts recommend the use of dexamethasone to decrease the inflammation and thus reduce the risk of airway compromise and improve antibiotic penetration.8 Complications, in addition to airway compromise, are mediastinitis, subphrenic abscess, pericardial/pleural effusion, empyema, abscess of the mandible, infection of the carotid sheath, rupture of the carotid artery, and thrombophlebitis of the internal jugular vein.7 The mortality rate exceeded 50% in the preantibiotic era but currently is reported to be fewer than 5%.5

Figure 2. Computerized tomography with contrast of the neck, showing a 1.3 0.9 cm hypodense area posterior to the symphysis of the mandible, likely representing a necrotic lymph node.

The differential of neck masses includes congenital, inflammatory, neoplastic, and traumatic processes. The most common are thyroglossal duct cysts, dermoid cysts, branchial cleft cysts, and cystic hygroma. Thyroglossal duct cysts can be recognized by their midline location between the hyoid bone and the suprasternal notch and by their upward movement with swallowing. Dermoid cysts are also in the midline and contain both solid and cystic components. A branchial cleft cyst feels smooth and fluctuant and is located along the lower anterior border of the sternocleidomastoid muscle. Cystic hygroma is a mutiloculated cyst that is diffuse, soft, and compressible and contains lymphatic fluid.

Lymphadenopathy accounts for the majority of neck masses in children and is most often of infectious etiology, either caused by a reaction to a local or systemic infection or involved in the infection itself. Causes of reactive lymphadenopathy in the cervical area are upper respiratory tract infections, pharyngitis, dental infections, and HIV. Pathogens that directly involve the nodes are mycobacterial organisms, cat scratch disease (Barlonella henselae), toxoplasmosis, and pyogenic bacteria (usually group A beta- hemolytic streptococci and Staphylococcus aureus).

The location of the lymphadenopathy may be helpful for differentiating the site of primary infection. Submandibular nodes are enlarged in diseases involving the oral cavity and the sinuses. Midjugular nodes are associated with hypopharyngeal and laryngeal infection while upperjugular nodes drain infection in the oropharynx and oral cavity. Supraclavicular nodes are usually involved in processes that originate below the clavicles including the lungs.

Sialoadenitis is most often caused by an infection of the parotid or submandibular glands but may result from reactive lymphadenopathy within the gland. Infections typically are associated with obstruction of the salivary flow due to dehydration, salivary stone, or sludge.

Tumors that present as neck masses include thyroid adenoma, lymphoma, thyroid cancer, metastases from squamous cell carcinoma, and adenocarcinoma. Neuroblastoma and leukemia are the most commontumors with cervical lymphadenopathy in patients less than 6 years of age. After 6 years, the most common tumor to present with cervical lymphadenopathy is Hodgkin’s lymphoma, followed by non- Hodgkin’s lymphoma and rhabdomyosarcoma.

In children, deep neck space infections are caused by acute tonsillitis with involvement of the peritonsillar space, whereas in adults they usually result from dental infections.4

Retropharyngeal abscesses (RPA) are infections in the space anterior to the prevertebral layer of the deep cervical fascia, most commonly seen in patients younger than 5 years of age because they have a greater number of lymph nodes in this region. In a retrospective review of patients with RPA,9 the most common presenting symptom was limitation of neck movement; 45% had limitation of neck extension, 36% had torticollis, and 12% had limited neck flexion. Other common presenting symptoms were the following: neck pain (38%), fever (17%), sore throat (17%), neck mass (16%), and respiratory distress or stridor (5%).

On examination, an asymmetric swelling of the posterior pharyngeal wall is highly suspicious of RPA, but such swelling may be absent in smaller children. RPA can also present as a neck mass. Streptococcus pyogenea, Staphylococcus aureus, and anaerobic bacteria are the most common etiologic agents. Radiographie findings include thickening of the retropharyngeal soft tissue (defined as more than 50% of the width of the vertebral body), loss of the normal cervical lordosis, and air in the retropharyngeal space. CT with contrast is the preferred diagnostic test. A chest radiograph should be performed as the RPA can extend into the mediastinum. Treatment should include parenteral antibiotics that are active against common oral facultative and anaerobic bacteria. The benefit of surgical drainage is determined by the maturity of the infection and the degree of airway compromise. Studies have shown that medical treatment alone can be curative in well over 50% of the patients.10,11 Late complications include rupture of the abscess with aspiration, asphyxiation, or pneumonia; empyema; and mediastinitis.9

REFERENCES

1. Hartmann RWJr. Ludwig’s angina in children. Am Earn Phys. 1999;60:109-112.

2. Weisengreen HH. Ludwig’s angina: historical review and reflections. Ear Nose Throat J. 1986;65:457-461.

3. Kurien M, MathewJ, Job A, et al. Lugwig’s angina. Clin Otolaryngol. 1997;22:263-265.

4. Nguyen VD, Potter JL, Hersh-Schick MR. Ludwig angina: uncommon and potientially lethal neck infection. AJNR. 1992;13:215-219.

5. Beasley DJ, Amedee RG. Deep neck space infections. / La State Med Soc. 1995;147:181-184.

6. Srirompotong S, Art-smart T. Ludwig’s angina: a clinical review. Eur Arch Oto-Rhino-Laryngol. 2003:1-4.

7. Barakate MS, Hemli (M, Jenscn MJ, et al. Ludwig’s angina: report of a case and review of management issues. Ann Otol Rhinol Laryngol. 2001;110:453-456.

8. Busch RF, Shah D. Ludwig’s angina: improved treatment. Otolaryngol Head Neck Surg. 1997; 117:S172-S175.

9. Lee SS, Schwartz RH, Bahadori RS. Retropharyngeal abscess: epiglottits of the new millennium. J Pediatr. 2001;138:435-437.

10. Craig FW, Schunk JE. Retropharyngeal abscess in children: clinical presentation, utility of imaging, and current management. Pediatrics. 2003;111:1394-1398.

11. Albright JT, Pransky SM. Nontuberculous mycobacterial infections of the head and neck. Pediatr Clin North Am. 2003;50:503- 514.

Copyright Westminster Publications, Inc. Apr 2005

Neonatal Hyperparathyroidism Due to Maternal Hypoparathyroidism and Vitamin D Deficiency: A Cause of Multiple Bone Fractures

Introduction

Neonatal hyperparathyroidism is a rare clinical entity with various etiologies. Neonatal hyperparathyroidism can be primary or secondary. secondary hyperparathyroidism can be due to maternal hypocalcemia, which may develop during hypoparathyroidism, pseudohypoparathyroidism, chronic renal failure, and renal tubular acidosis.1’6 Neonatal hyperparathyroidism can be fatal or can also result in several sequelae. Intrauterine bone fracture is a rare complication of neonatal hyperparathyroidism.1 In addition, severe respiratory involvement is also encountered in neonatal hyperparathyroidism.

We present a case of neonatal hyperparathyroidism with multiple bone fractures and findings of rickets due to maternal hypoparathyroidism and vitamin D deficiency.

Patient Report

A 3-month-old female infant was referred to this university hospital because of respiratory distress and multiple bone fractures. She was born to a 25-year-old woman by noncomplicated vaginal delivery at 36 gestational weeks. She was the third sibling of the family; she had a healthy brother and sister aged 7 and 4 years, respectively. Her parents were first-degree cousins. Her mother had been suffering recurrent carpopedal spasms for the last 4 years including the pregnancy period but she did not receive any therapy for hypocalcemia.

In her first month of life, the patient failed to gain weight and suffered from recurrent apnea and cyanosis with respiratory distress. When she was 1 month old, she was hospitali/ed at a local community hospital. She was diagnosed with pneumonia and treated with nonspecific parenteral antibiotics. It was also noticed that she had multiple bone fractures with significant deformities on extremities suggesting congenital rickets. At that time serum calcium, phosphorus, alkaline phosphatase (ALP) and 25 hydroxyvitainin D levels were in normal ranges; however, parathyroid hormone (PTH: 98.7 pg/mL, normal: 13-66 pg/mL) was elevated. She was administered 800 U of cholecalciferol per day. After 1 month of hospitalization, there was no improvement in the pulmonary involvement and she was referred to this clinic.

At 3 months of age, her weight was 2,400 g, height 47 cm, head circumference 37 cm. She was tachypneic (respiration rate 60-80 breaths/minute), her heart rate was 166 beats/minute, and axillary body temperature 36.7C. There was significant loss of subcutaneous tissue and decreased muscle tone. On pulmonary examination, subcostal and intercostal retractions were noticed. On chest auscultation, bilateral, widespread, fine crackles with wheezing were present. Enlargement of costochondral junctions and thickening of wrists were detected. There were also deformities on upper and lower extremities.

Results from laboratory examination were as follows: hemoglobin: 10.3 g/dL; hematocrit: 31.8%; leukocytes: 9,000/mm^sup 3^; platelets: 297,000/mm^sup 3^; calcium: 9.3 mg/dL; phosphorus: 4.8 mg/ dL; ALP: 431 U/L (normal:35-462); arterial blood pH: 7.24; bicarbonate: 29.7 mmol/L; partial pressure of oxygen (Po^sub 2^): 45.6 mm Hg; partial pressure of carbon dioxide (PcO^sub 2^): 69.6 mm Hg: O2 saturation: 72.9%; intact PTH: 160 pg/mL (normal 12-65 pg/ mL); 25-hydroxyvitamin D: 35 ng/mL (normal:10-40 ng/mL); sweat chloride test: 7 mEq/L. Chest radiography and thoracic computed tomography (CT) revealed atelectasis on the left lung and compensatory hyperaeration on the right lung. Anterior ends of the ribs were enlarged in the chest radiograph as well. Serum quantitative immunoglobulins G, M, and A were within normal levels. A barium esophagogram showed no gastroeosophageal reflux. An echocardiogram revealed mild mitral insufficiency. Nasopharyngeal aspirate was tested for RSV PCR and found to be positive. Radiographs of the extremity showed multiple fractures at bilateral proximal humerus, distal radius, and distal femur with severe demineralization. There was also clipping, fraying, and widening of metaphysis at the distal ends of radius, humerus, and femur (Figure 1).

Her mother was investigated for overt tetany. The serum concentration of calcium was 4.6 mg/dL; phosphorus: 10.4 mg/dL; ALP: 79 U/L; intact PTH:

Figure 1. The radiologie view of extremities showing multiple fractures at proximal humerus, distal radius, and distal femur bilaterally with severe demineralization and cupping, fraying, and widening of metaphysis at the distal ends of radius, humerus, and femur.

In the follow-up, the patient was treated with intravenous antibiotics, nebulized salbutamol, supplemental oxygen, and chest physiotherapy. Maintenance doses of vitamin D (400 ILJ/day) were administered perorally. Daily 500 mg elemental calcium requirement was provided by enterai feeding. After 5 weeks of intensive care, the clinical picture of severe respiratory distress subsided and atelectasis of the left lung improved. Callus formation on the sites of fracture and recovery in the metaphyses were observed. Intact PTH level decreased to 64 pg/mL and no more fractures were noted.

Discussion

The patient in our case suffered from neonatal hyperparathyroidism due to maternal hypoparathyroidism associated with vitamin D deficiency. She had severe multiple intrauterine bone fractures leading to deformities and respiratory failure. Neonatal hyperparathyroidism may result from several diseases related to maternal hypocalcemia. Maternal hypocalcemia causes reduced maternal- fetal calcium transfer and subsequently fetal hypocalcemia and hyperparathyroidism. One of the main causes of maternal hypocalcemia is hypoparathyroidism. In fact hypoparathyroidism in pregnancy leads to fewer symptoms of hypocalcemia. Serum calcium concentrations of these women were found to be higher than in the preconceptual period. Increases in 1,25-dihydroxyvitamin D and intestinal calcium absorption are suggested to be the reasons for a lower calcium and calcitriol requirement and fewer symptoms of hypocalcemia in maternal hypoparathyroidism during pregnancy.7 Nevertheless, the mother of our patient had vitamin D deficiency in addition to hypoparathyroidism. Vitamin D deficiency may impede a rise in 1,25 hydroxyvitamin D level leading to severe hypocalcemia. Although the vitamin D level was low in the mother it was found to be in normal range in our patient. The fetus is thought to be entirely dependent on the mother for its supply of 25-hydroxyvitamin D, which is believed to cross the placenta.8 The normal vitamin D level in our patient can be explained by sufficient vitamin D supplementation since birth.

Striking pulmonary abnormalities have been described in rickets. Lobar and segmentai atelectasis, compression atelectasis under the internal projections of the rachitic rosaries at the costocondral junctions, and interstitial pneumonitis are some of these changes. Besides, as most children with rickets have malnutrition, they tend to have recurrent pulmonary infections.9 Our patient’s atelectasis, persistent pneumonia, and hypoxia can be described by her rickets- like chest deformities.

The duration and severity of neonatal hyperparathyroidism secondary to maternal hypoparathyroidism are variable. Some patients with neonatal hyperparathyroidism can be asymptomatic without any biochemically or radiologie findings. However, maternal hypoparathyroidism can result with spontaneous abortions, stillbirth, and neonatal deaths.1,10,11 The range of calcium levels in these patients also varies. Serum PTH levels in neonatal hyperparathyroidism generally normalize in a short period of time. Long-head et al1 reported 2 infants with congenital hyperparathyroidism, in 1 of whom serum PTH concentrations were normalized just after birth, and in the other, 12 days after delivery. Contrarily, another patient with neonatal hyperparathyroidism and vitamin D deficiency had high PTH levels for 3 months postnatally.2 Vitamin D deficiency in this patient probably contributes to persistent hyperparathyroidism. Our patient also had high PTH levels for approximately 4 months. The reason for prolonged hyperparathyroidism and the Findings of active rickets on plain radiographs were attributed to insufficient calcium supplementation and undernutrition in this patient.

Neonatal hyperparathyroidism due to maternal causes can result in severe life-threatening complicadons. Follow-up during pregnancy is the most important preventive approach while early diagnosis and appropriate management of the affected infant is essential for avoiding morbidity and mortality.

REFERENCES

1. Loughead JL, Mughal Z, Mimouni F, et al. Spectrum and natural history of congenital hyperparathyroidism secondary to maternal hypocalcemia. AmJPmnatol. 1990:7:350-355.

2. Sann L, Thomas DA, Frederich A, et al. Congenital hyperparathyroidism and vitamin D deficiency due to maternal hypoparathyroidism. Ada Pediatr Scand. 1976;65:381-385.

3. Vidailhet M, Monin P, Andre M, et al. Neonatal hyperparathyroidism secondary to maternal hypoparathyroidism. Arch Fr Pediatr. 1980; 37:305-312.

\4. Glass EJ, Barr DCi. Transient neonatal hyperparathyroidism secondary to maternal pseudohypoparathyroidism. Arch Dis Child. 1981;56:565-568.

5. Savani RC, Mimouni F, Tsang RC. Maternal and neonatal hyperparathyroidism as a consequence of maternal renal tubular acidosis. Pediatrics. 1993;91:661-663.

6. Levin TL, States L, Greig A, Goldman HS. Maternal renal insufficiency: a cause of congenital rickets and secondary hyperparathyroidism. Pediatr Radial. 1992:22:315-316.

7. Kovacs CS, Kronenberg HM. Maternalfetal calcium and bone metabolism during pregnancy, puerperium and lactation. Endocr Rm. 1997; 18:832-872.

8. Salle BL, Delvin EE, Lapillonne A, et al. Perinatal metabolism of vitamin D. Am/amNute2000;71:1317S-1324S.

9. Khajaui A, Amirhakimi GH. The rachitic lung. Pulmonary finding in 30 infants and children with malnutritional rikets. Clin Pediatr. 1977;16:36-38.

10. Eastell R, Edmonds CJ, de Chayal RC, McFadyen IR. Prolonged hypoparathyroidism presenting eventually as second trimester abortion. Br MedJ. (Clin Res Ed) 1985:291:955-956.

11. Anderson GW, Musselman L. The treatment of tetany in pregnancy. Am J Obstet Gynecol. 1942:43:547-567.

AyferAlikasifoglu, MD1

E. Nazli Gone, MD1

Ebru Yalcin, MD?

Deniz Dogru, MD2

Nursen Yordam, MD1

Clin Pediatr. 2005;44:267-269

Hacettepe University, Divisions of1 Pediatrie Endocrinology and 2Pulmonary Medicine, Ankara, Turkey.

Reprint requests and correspondence to: Ayfer Alikasifoglu, MD, Associate Professor of Pediatrics, Hacettepe University, Division of Pediatrie Endocrinology, Ankara, Turkey 06100.

2005 Westminster Publications, Inc., 708 Glen Cove Avenue, Glen Head, NY 11545, U.S.A.

Copyright Westminster Publications, Inc. Apr 2005

Researchers Link POPs to Respiratory Diseases

Researchers at the University of New York at Albany have found a link between respiratory dis eases and New York state residents who live in or near hazardous waste sites containing persistent organic pollutants (POPs). Their report was published in the December 2004 issue of Environmental Toxicology and Pharmacology (vol. 18, issue 3, available at ScienceDirect.com).

The researchers analyzed hospitalization statistics in 1595 state zip codes – 213 (with a 2000 Census population of some 2.8 million) containing or abutting a hazardous waste site containing POPs, including polychlorinated biphenyls (PCBs) and persistent pesticides, and 1382 (with a 2000 population of 4.7 million) identified as clean sites. The POP-contaminated sites were identified by the U.S. Environmental Protection Agency, the New York Department of Environmental Conservation, and the International Joint Commission, which advises the U.S. and Canadian governments on issues relating to boundary waters.

To eliminate other factors that contribute to respiratory diseases, such as income, excess smoking, and lack of exercise, the researchers also studied a subset – 78 zip codes – of residents living in or near POP-contaminated sites along the Hudson River, from Hudson Falls to Manhattan. This area has fewer smokers, higher per capita income, and better diet and exercise habits than much of the rest of the state. Researchers discovered that residents of this area were hospitalized for respiratory infections more often than people not living in or near POP-contaminated sites.

Chest x-rays such as this illustrate the damages of respiratory disease.

Altogether, researchers found that hospitalization rates due to chronic bronchitis and other infectious respiratory diseases were about 20% higher for residents of POP-related zip codes than for the general New York state population. Specifically, results showed statistically significant increases in pneumonia, influenza, and chronic bronchitis in men and women aged 45 to 74, and in unclassified chronic airway obstructions in men and women over 45.

“These observations show us that the higher frequency of respiratory dis ease cannot be explained by the usual suspects of bad diet and smoking,” said David O. Carpenter, one of the study authors and director of the university’s Institute for Health and the Environment. “It strengthens our hypothesis that populations living by the Hudson are breathing in PCBs, which causes their immune systems to malfunction, leading to more infections.

“It is usually thought that exposure to POPs comes primarily from eating contaminated fish and other animal products, but our observations cannot be explained by different patterns of ingestion,” Carpenter said. “Our results suggest that simply living near a contaminated site increases the risk of exposure to POPs, and that this increases the risk of infections as a result of suppression of the immune system.”

In an earlier report, Carpenter showed that hospitalization for five infectious childhood diseases was 30% greater in POP- contaminated areas than in clean zip codes. And another study shows that Dutch infants exposed to dioxins and PCBs have elevated incidence of recurrent middle-ear infections and chicken pox, and a lower prevalence of allergic reactions [Weisglas-Kuperus et al. (2000) Environmental Health Perspectives 108: 1203-1207].

For more information, contact Carpenter at [email protected]. edu.

Copyright Water Environment Federation Apr 2005

Obesity Creates Need for Oversized Caskets

BIRMINGHAM, Ala. — When the funeral director saw the fat man in the small town, they engaged in some friendly banter about death. “You’d tell him, ‘You’re going to have to go on a diet. You’ve got to lose some of that weight,” said John C. Rudder, owner of Rudder Funeral Home in Scottsboro.

“And he’d say ‘Yeah, I know, you ain’t got a box big enough to fit me.’ “”And we didn’t,” Rudder said.

The solution when the man died: Order an oversized casket.

With an increasing number of Americans considered obese – including many in Alabama – funeral directors have been dealing with a big problem. Their caskets were not large enough.

Enter companies like Southern Heritage Casket Co. in Oxford, about 60 miles east of Birmingham. It’s one of many firms across the nation that are pumping up the size of caskets to meet the needs of increasingly large people.

Last fall, the health advocacy group Trust for America’s Health ranked Alabama the most obese state in the nation. Twenty-eight percent of adults in the state were classified as obese.

An additional 35 percent of adults 18 and older are overweight in Alabama, according to the state Department of Public Health.

Obesity causes about 400,000 deaths in the United States every year, according to Trust for America’s Health. It’s poised to overtake tobacco use as the leading cause of preventable death.

And handling funeral arrangements for an obese person can create additional stress for the family and for the funeral professionals involved.

For years, caskets were built with a standard inside shoulder width of 22 inches to 24 inches, said Jeff Cheek, president of the Southern Heritage, which only makes steel caskets. Now, the company builds standard caskets with widths up to 26 inches and a line of oversized caskets with interior widths from 28 inches to 44 inches.

The caskets are welded together, sanded and painted at Southern’s Heritage’s plant in Oxford.

“It was almost unheard of to sell stuff this big 10 or 15 years ago,” Cheek said.

Cheek, 45, and his father, Arvel Cheek, left another casket company to start Southern Heritage in the 1980s. They started as specialists in children’s caskets, which they still make in small quantities.

Jeff Cheek said the company started making oversized caskets in the mid-1980s and began offering the 40- to 44-inch caskets only about two years ago. Southern Heritage had to buy new paint-drying ovens to accommodate the larger size and is planning for more retrofitting of the factory to streamline the process.

Oversized caskets now account for about 20 percent of the company’s sales, Jeff Cheek said. The biggest caskets still make up a small proportion of the roughly 50 caskets the company makes per day.

“The 40s and above is probably just now getting to the point that it’s one a day,” he said.

He said said Southern Heritage has been able to get ahead of other casket companies by offering a selection of oversized styles instead of big, plain boxes. Now the company’s catalog offers big caskets in a variety of colors and decorations.

“I realize that everything that leaves here goes to someone who has lost a loved one,” he said. “That was the main thought behind us expanding the selection on the big ones. Why is it if someone’s obese that they’re not entitled to options like everyone else?”

The company ships its caskets throughout the South and to Ohio and Oklahoma. Jeff Cheek said he doesn’t notice geographical differences in demand for oversized caskets, but said demand spikes during periods of extreme hot and cold that strain the bodies of obese people.

“It’s all over. We have an epidemic,” he said. “I can’t be judgmental. I’d like to be 25 pounds lighter than I am. I’m sure anyone who’s overweight would rather not be that way.”

Rudder, the funeral director in Scottsboro, described what happened when the obese man he used to joke with died in September from congestive heart failure. Rudder said the 700-pound man was to be put in an above-ground mausoleum with his parents.

“This fellow had gotten so large that the casket that he had to accommodate his size didn’t fit inside the mausoleum,” he said. The man was buried outside.

There can be other logistical problems when an extremely large person dies – a large casket might not fit into the hearse or might not be able to fit into a chapel.

Costs can be higher, too. When an obese man died in July 2004, his family paid $3,250 for his casket alone, $600 more than it would have cost for a regular casket, Rudder said.

Rudder said there is additional demand for oversized caskets because of an increasing number of people who are simply large, not overweight.

“My youngest son is 6-feet-4 and will weigh 250 pounds,” he said. “And he’s a big boy. He really won’t fit inside a standard size casket. But you don’t think of him of being obese. He’s big.”

Bigger caskets affect cemeteries, too.

Mike Hauser is marketing director of the Ridout’s funeral homes and cemeteries in the Birmingham area. He said newer parts of the company’s cemeteries now are laid out with wider spaces for graves to accommodate larger bodies and for the use of vaults – containers that go around the bodies and prevent soil settling.

In the case of an extremely large casket and vault, a family that purchased a family plot might simply allow the grave to take up two spaces rather than one, he said.

In the meantime, Rudder and other funeral directors are asking for bigger caskets.

“For the last several years, whenever we can get anybody to listen to us, we’ve been telling the casket manufacturers that they need to give us caskets to accommodate these people where they don’t look like they’re a cork in a bottle,” he said.

The casket companies have responded.

Last fall, Indiana-based Batesville Casket Co., one of the nation’s largest casketmakers, introduced 13 new oversized models and introduced the Dimensions brand. It now offers a total of 53 oversized models, company spokesman Joe Weigel said.

And Lynn, Ind.-based Goliath Casket Co. has continued to increase the size of its offerings.

“We make very large oversize caskets. Oversized is kind of an understatement. They’re ‘supersized,’ to coin a famous term,” said Keith Davis, who owns the company with his wife, Julane Davis.

Sales have doubled in the last month, he said. The company could sell 800 caskets this year, and it recently rolled out a 52-inch casket.

“That is a little bit wider than a standard pickup bed size,” he said.

“The 44-inch, 48-inch, 52-inch are for body weights between 650 and 1,200 pounds. There are people that large, believe it or not,” he said.

There are extra supports to make sure the weight doesn’t cause the casket to break.

Information from: Birmingham Post-Herald

Inflammatory Myofibroblastic Tumor of the Central Nervous System: Clinicopathologic Analysis of 10 Cases

Abstract

To verify the pathologic features, anaplastic lymphoma kinase (ALK) expression and biologic behavior of inflammatory myofibroblastic tumors (IMTs) of the central nervous system (CNS), we analyzed 10 cases of IMTs-CNS (8 cranial, 1 spinal, and 1 orbital). Our series of IMTs of the CNS showed a male predominance (male:female = 6:4) and a wide age range (10-60 years; mean age, 46.7 years). Lesion location also varied, but they were basically dura-based. Radiologically, they showed two patterns: isolated mass forming (n = 6) and an en plaque-like pattern (n = 4). Histopathologically, plasma cell granuloma (PCG)-like (n = 5) or fibrohistiocytic (FHC) variant (n = 5) was present. No correlation was found between the radiologie and histopathologic patterns. Spindle-shaped mesenchymal cells of IMTs expressed smooth muscle actin (SMA) in all cases. ALK expression was not found in our IMTs of the CNS. Late recurrence was found in 2 cases in different sites (20%). Pathologically, IMT-CNS could be subclassified into PCG-like and FHC. Immunostaining for SMA was found to helpfully discriminate myofibroblastic cells and to make a differential diagnosis. Although our cases did not show ALK immunoreactivity, some IMTs-CNS can recur, which suggests the neoplastic potential of these tumors. The rearrangement of the ALK gene in IMTs-CNS should be verified by an examination of more cases.

Key Words: Anaplastic lymphoma kinase, Central nervous system, Inflammatory myofibroblastic tumor, Inflammatory pseudotumor. Plasma cell granuloma.

INTRODUCTION

Inflammatory myofibroblastic tumor (IMT) is a lesion characterized by the proliferation of myofibroblastic spindle cells with mixed inflammatory infiltrates of plasma cells, lymphocytes, eosinophils, and histiocytes. The constituent inflammatory cells are mature and polyclonal, and occur in virtually every organ system, including the liver, mesentery, gastrointestinal tract, retroperitoneum, urinary bladder, upper respiratory tract, and mediastinum (1, 2), as well as in the lung, the originally described and most common site. Because of the wide spectrum of its histologic and clinical features, this tumor has been referred to variously as “inflammatory pseudotumor,””plasma cell granuloma,””pseudosarcomatous myofibroblastic proliferation,””inflammatory myofibrohistiocytic proliferation,” etc. In the 2002 World Health Organization classification of soft tissue tumors, these lesions were renamed “inflammatory myofibroblastic tumors” and allocated to the soft tissue tumor category (3). Recently, chromosomal translocations involving the ALK gene were reported in about 35% to 50% of IMTs, and resultant ALK protein overexpression in myofibroblastic cells was found in 35% to 60% of IMT cases, suggesting neoplasm of these tumors rather than a reactive or reparative process (4-6). However, the concept of the same lesions in the central nervous system (CNS) was not changed and was still referred to as “plasma cell granuloma” or “inflammatory pseudotumor” but rarely as “inflammatory myofibroblastic pseudotumor” when myofibroblasts were rich in the mass. These different nosologies for the same kind of tumor inevitably cause confusion during the diagnosis and management of these lesions. We consider that they are the same lesion that occurs in the CNS and suggest that they be referred to in unison as IMT. The CNS, including the spinal cord, may be one of the rare sites affected by IMTs, and if we included the lesions described using the various terminologies above, some 100 sporadic cases have been reported in the literature (7, 8). To verify the clinicopathology, ALK expression, and biologic features of IMT-CNS, we analyzed clinicopathologic features and performed ALK immunohistochemistry on 10 cases of IMTs-CNS.

MATERIALS AND METHODS

Ten cases of IMTs-CNS (8 intracranial, 1 spinal, and 1 intracranially extended orbital case) were selected from the archives of three referral hospitals: Seoul National University Hospital, Samsung Medical Center, and Inje University Hospital. The patients presented between 1996 and 2003. Clinical histories and radiologic findings were obtained from medical records. As a control for the ALK immunohistochemical study, 10 cases of extra-CNS and extra-orbital IMTs were included. The IMTs-CNS group was composed of 6 males and 4 females, and patients’ ages ranged from 10 to 65 years with a mean age 46.7 years. Ten cases of extracranial IMTs were used as controls for immunohistochemical study. Extracranial IMTs were located in the lung (n = 3), urinary bladder (n = 2), liver (n = 2), and one in each of colon, chest wall, and retroperitoneum; patients’ ages ranged from 5 to 72 years with a mean of 31.1 years. Three cases of cerebral and spinal idiopathic hypertrophie pachymeningitis were included to compare smooth muscle actin (SMA) immunoreactivity with that of IMTs-CNS.

Two pathologists independently reviewed the hematoxylin and eosin slides and agreed to a diagnosis of IMT. Immunohistochemical staining was performed using a conventional labeled streptavidin- biotin-peroxidase method (LSAB Kit, DAKO, Glostrup, Denmark) according to the manufacturer’s protocol. Briefly, 4-m tissue sections were placed on silanecoated slides, deparaffinized, and rehydrated. After antigen retrieval by microwaves blocking endogenous peroxidase activity with hydrogen peroxide, goat serum (DAKO) to prevent nonspecific reaction and primary antibodies were applied sequentially. The primary antibodies used were as follows: vimentin, SMA, epithelial membrane antigen (EMA), Ki-67, p53, ALK, CD3, CD5, CD10, CD20, and kappa-, and lambda-light chains, Tdt, S- 100 protein, c-kit, CD21 (all from DAKO), cyclin D1 (Novocastra, Newcastle-upon-Tyne, UK and p16 (Pharmingen, San Diego, CA), The slides were incubated in biotinylated goat anti-mouse/rabbit immunoglobulin and then in a solution of streptavidin-biotin complex. Immunoreactivity was visualized by using 3,3- diaminobenzidine. Tonsil, intestine, and ALK-positive anaplastic large cell lymphoma tissues were used as appropriate positive control and as an antibody control primary antibodies were omitted.

RESULTS

Clinical and Radiologic Features

The clinical and radiologic features of the 10 IMTs-CNS patients are summarized in Table 1. Tumor locations varied and included the supratentorial convexity, including the falx (n = 5), infratentorial convexity (n = 2), anterior cavernous sinus (n = lumbar spine (n = 1), and the orbit (n = 1). All intracranial tumors, except for one cavernous sinus mass (Patient 5), occurred as dura-based meningeal lesions with or without brain involvement (4 cases with cerebral parenchymal invasion and 2 cases with cerebellar parenchymal invasion), and no example of an isolated intraaxial tumor was presented. Radiologically, IMTs-CNS showed two different types of growth pattern, an isolated mass-forming type and en plaque-like type, which were accompanied by leptomeningeal thickening with or without infiltration into the brain parenchyma or perilesional brain edema (Fig. 1). In enhancing studies, all lesions were well enhanced except Patient 7, which showed multifocal enhancement. A case of orbital IMT presented as a mass-forming lesion with intracranial extension and the fibrohistiocytic (FHC) variant (Fig. 1). Four cases had synchronously multiple occurrences in intracranial sites, and the remaining 6 cases had a single lesion (Fig. 1). Combined orbital and mastoid involvements were found in 20% of our cases. All patients initially presented with neurologic manifestations depending on the location of tumor but were without constitutional symptoms. Laboratory data were normal in all except for an elevated erythrocyte sedimentation rate in Patient 2. Patient 3 had a temporal lesion and PCR for herpes simplex virus in cerebrospinal fluid was negative. Two cases (Patients 1 and 7) experienced intracranial recurrences in sites other than the original location; 1 recurred twice in the right mastoid and in the right cerebellar tentorium, with cerebellar parenchymal involvement 12 and 15 years after initial complete resection of the right orbital IMT and despite radiotherapy. The other case recurred in the infratentorial convexity 7 years after resection of a mastoid tumor in the same side. The histologies of the recurred tumors were identical to those of the primary tumors.

Pathologic Features

Like IMTs arising in other organs, IMTs-CNS showed a wide spectrum of histologic features. Depending on the predominant histology, IMTs-CNS could be classified into two types: the so- called plasma cell granuloma (PCG)-like type or the FHC variant (Tables 1 and 2; Fig. 2). The PCG-like type (Patients 1, 3, 4, 6, and 9) was characterized by predominant lymphoplasma cell infiltrations in vascular stroma with a minor component of myofibroblastic cells. Within the PCG-like type, the compositions of inflammatory cells varied. Plasma cells occasionally had two nuclei and an immature appearance. However, the CD79a-positive plasma cells were polytypic populations and expressed both κ and λ light chains. Patients 1, 3, 6, and 9 exhibited extensive lymphocytic infiltrations into the brain parenchyma through Virchow- Robin spacesas well as the leptomeninges. These 4 cases with both leptomeningeal and brain involvement were difficult to distinguish from lymphoproliferative disorders or infectious conditions. In Patient 3, the lymphoid cells were mainly small B cells with no atypism and low mitotic activity. They were heterogeneously positive for lymphoid markers, CD3 or CD20, but negative for CD10, Tdt, and CD5. The spinal IMT case (Patient 4) showed infiltrates of various inflammatory cells and vague granulomas.

FHC variant (Patients 2, 5, 7, 8, and 10) was characterized by distinct myofibroblastic proliferation with a minor population of mixed inflammatory cells. The myofibroblastic cells had oval nuclei with no pleomorphism and small indistinct or distinct nucleoli (Fig. 2). The hyalinization of stroma was variable from case to case.

As summarized in Table 2, SMA was positive in the most fibroblastic cells of all CNS and extra-CNS IMTs (control cases), but SMA positivity was absent or very rarely found in fibroblastic cells of the cerebral and spinal pachymeningitis (Fig. 2). ALK- positive cells were not found in any IMTs-CNS; however, 20% of the extra-CNS IMTs revealed ALK expression (2 of 10 cases) (Fig. 2). EMA, CD21, and c-kit were all negative in CNS- and extra-CNS IMTs; these were undertaken to rule out meningioma, follicular dendritic cell tumor, and stromal or embryonal tumor, respectively. No aberrant p53 expression was found in any IMTs-CNS. The Ki67 labeling index in infiltrating lymphocytes was moderately high (about 5%) but in myofibroblasts was low (

DISCUSSION

IMTs are enigmatic tumors that may involve virtually every organ but rarely originate in the CNS. IMT-CNS is more commonly called an “inflammatory pseudotumor,” which represents a simple reactive or benign inflammatory process. However, the biologic, pathologic, and clinical features of IMT-CNS have not been well established. Molecular genetic and immunohistochemical studies for ALK are extremely rare in IMT-CNS (7, 9).

TABLE 1. Clinical, Radiologic, and Histologic Features of IMTs- CNS

According to a review of 57 cases of inflammatory pseudotumors of the CNS by Hausler et al, they arise predominantly from dural/ leptomeningeal structures (60%) (7). They commonly manifest as intracranially and extra-axially with or without brain parenchymal involvement, or rarely as pure intraaxial (12%) or intraventricular lesions (12%). They also may extend from intracerebral to extracerebral sites (9%). Based on a review of 38 intracranial and spinal IMTs by Buccoliero et al, the maleifemale ratio was 7:3 and median age was 32 years, ranging from 5 to 76 years. Of these 38 cases, 82% presented as a single intracranial lesion, 3% as a solitary spinal lesion, and 16% as multiple synchronous or metachronous lesions (10). One patient with synchronous multiple IMTs showed involvement of the cerebral, cerebellar, brainstem, and spinal leptomeninges (11), and another patient with dual IMTs located in the spinal meninges and the cerebral falx (12). Even coexisting IMTs in the lung and CNS have been reported (13). The recurrence rate has been reported to be as high as 40% within 2 years, in general, especially following incomplete tumor resection (7, 14).

FIGURE 1. The multiple en plaque type (Patient 1) showing leptomeningeal thickening with enhancement and edema in the surrounding brain. (A) Contrast-enhanced, T1-weighted axial MR image. (B) T2-weighted axial MR image. (C) T2-weighted axial MR image of orbit. (D) Mass forming type; CT (Patient 5) exhibiting a wellenhanced, round mass (arrow) in the right cavernous sinus.

The present study shows that IMTs-CNS can arise everywhere in the CNS including the spinal cord but that they occur as dura-based, mass-forming lesions (60%) or en plaquelike lesions (40%). It also shows a male dominance (male: female = 6:4) and a mean age in the mid-fifth decade. All cases appeared as homogeneously well-enhanced lesions on MRI except 1 case that showed heterogeneous enhancement and an intratumoral hemorrhage. Associated orbital or mastoid involvements by IMTs-CNS were not uncommon; each was found in 20% of cases in the present study, which were synchronously or metachronously presented. Brain parenchymal infiltration was higher in our series (60% of cases) than that of Hausler et al review (16% of cases) (7).

Histopathologically, IMTs were classified in several ways by Coffin et al ( 1 ). They described three major histologic patterns, which depended on location. In nonpulmonary IMTs, they were of a myxoid/vascular pattern, a compact spindle cell pattern, and a hypocellular fibrous pattern; whereas in pulmonary IMTs, they were of an organizing pneumonia pattern with central hyalinization, a fibrous histiocytoma-like pattern, and a lymphoplasmacytic pattern. We modified the Coffin et al classification of pulmonary IMTs into a two-tiered classification according to predominant features, i.e. PCG-like type and FHC variant. The latter is characterized by distinct myofibroblastic proliferation with a minor inflammatory cell component, and the former as the reverse, i.e. by a distinct inflammatory cell component with slight myofibroblastic proliferation. The schema of nonpulmonary IMTs by Coffin et al is not applicable to IMT-CNS because myxoid/vascular or hypocellular fibrotic patterns were not found in our series, although it should be added that our study included a limited number of cases. Even though our series could be divided into two subtypes, a mixed pattern composed of relatively equal proportion of PCG-like and FHC component exists.

The histopathologic diagnosis of IMTs-CNS is not always easy. Important differential diagnoses might be intracranial plasmacytoma, lymphoma or lymphoproliferative disorder, idiopathic hypertrophic pachymeningitis (15), and a lymphoplasma cell-rich variant of meningioma. Myofibroblasts are spindle mesenchymal cells that have ultrastructural features in common with smooth muscle cells and fibroblasts. The confirmation of the presence of SMA-positive myofibroblasts is potentially important for the differential diagnoses from idiopathic hypertrophie pachymeningitis or meningioma because we also confirmed the lack of or very rare SMApositive myofibroblasts in 3 cases of pachymeningitis (Fig. 2) and meningiomas (data not shown). In addition, idiopathic hypertrophic pachymeningitis usually shows unique abortive granulomas with central basophilic necrosis (or smudging) and contains multinucleated but small giant cells and some neutrophilic infiltration, which were not found in our IMTsCNS (15). Unlike the lymphoplasma cell-rich variant of meningioma, there are neither meningothelial proliferation with whorls nor EMA expression in IMTs- CNS. The infiltration of polyclonal plasma cells and other mixed inflammatory cells is a helpful feature when differentiating IMT- CNS and lymphoma or plasmacytoma. However, in cases having IMTs-CNS with parenchymal involvement, it was difficult to establish a correct diagnosis, and in such cases, molecular studies may verify the polyclonality of infiltrating lymphoid cells. We also had diagnostic difficulties, and 1 case was initially misdiagnosed as plasmacytoma.

FIGURE 2. Plasma cell granulomalike type (Patient 9). (A) Predominant lymphocytic infiltrations with scant myofibroblastic cells (hematoxylin and eosin, original magnification = 100). (B) Underlying brain parenchymal involvement (hematoxylin and eosin; left, original magnification = 100; right, original magnification = 200). (C) Fibrohistiocytic variant (Patient 2) (hematoxylin and eosin; original magnification = 200). (D) Abundant SMA-positive myofibroblastic cells in fibrohistiocytic variant (left panel, Patient 2) (immunoperoxidase stain; original magnification = 200). Only scattered SMA (+) cells in the plasma cell granuloma-like type (right panel, Patient 3) (immunoperoxidase stain; original magnification = 400). (E) The spindle cells of the idiopathic hypertrophic pachymeningitis do not express SMA (immunoperoxidase stain; original magnification = 200). (F) ALK is not expressed in the cranial IMT (left panel, Patient 7) (immunoperoxidase stain; original magnification = 200). ALK is positive in the cytoplasm of myofibroblastic cells in the IMT of the urinary bladder (right panel, control case) (immunoperoxidase stain; original magnification = 200).

TABLE 2. Immunohistochemical Findings

Despite an apparently benign morphologic pattern, evolving evidence strongly suggests the neoplastic nature of IMTs, including the translocation and clonal gene rearrangement of anaplastic lymphoma kinase (ALK) gene on the chromosome 2p23, and biologic behaviors such as local recurrences, and rare distant metastases, even malignant transformation (16-20). In cases of IMTs-CNS, the recurrence rate was reported to be 12.5% to 40% (8).

The tropomyosin genes TPM3 and TPM4 (20), the clathrin heavy chain gene CLTC (21 ), the cysteinyl-tRNA synthetase gene CARS (22), and the Ran-binding protein 2 gene RANBP2 (23) have been identified as fusion partners of the ALK gene. There have been only a few sporadic case reports of ALK overexpression in IMTs-CNS; one was a frontal tumor (9) and the other was a spinal tumor (7). Interestingly, both suffered from multiple local recurrences. However, in our study, which is the largest series to study the immunohistochemical expression of ALK in IMTs-CNS, no ALK-positive cell was found in IMTs-CNS. However, of the control cases of the extracranial and extra-orbital IMTs, 20% expressed ALK. Coffin et al reported that the overexpression of ALK was most frequent in abdominal and pulmonary IMTs in the first decade of life and that this is associated with a higher frequency of recurrence (5). A single case of IMT-CNS that underwent malignant transformation has been reported (7). In our series, the histology of recurred tumors was the same as that of the original tumors. \Our study indicates that ALK-expression in IMT-CNS is rare, and it is possible that some other discrete biologic subset identical to neoplastic IMT is included in the category “IMT-CNS,” including “inflammatory pseudotumors” or “plasma cell granulomas of the CNS.” Close clinical follow-up is recommended, especially if surgical excision is incomplete, because recurrence may occur even 10 years later. The rearrangement of the ALK gene in CNS and orbital IMTs should be verified in more cases.

REFERENCES

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2. Coffin CM, Humphrey PA, Dehner LP. Extrapulmonary inflammatory myofibroblastic tumor: A clinical and pathologic survey. Semin Diagn Pathol 1998;15:85-101

3. Fletcher CDM, Unni K, Mertens F. Pathology and Genetics, Tumors of Soft Tissue and Bone. World Health Organization Classification of Tumors. Lyon, France: IARC Press, 2002:91-93

4. Cook JR, Dehner LP, Collins MH, et al. Anaplastic lymphoma kinase (ALK) expression in the inflammatory myofibroblastic tumor: A comparative immunohistochemical study. Am J Surg Pathol 2001;25:1364- 71

5. Coffin CM, Patel A, Peking S, et al. ALK1 and p80 expression and chromosomal rearrangements involving 2p23 in inflammatory myofibroblastic tumor. Mod Pathol 2001;14:569-76

6. Cessna MH, Zhou H, Sanger WG, et al. Expression of ALK1 and p80 in inflammatory myofibroblastic tumor and its mesenchymal mimics: A study of 135 cases. Mod Pathol 2002;15:931-38

7. Hausler M, Schaade L, Ramaekers VT, et al. Inflammatory pseudotumors of the central nervous system: Report of 3 cases and a literature review. Hum Pathol 2003;34:253-62

8. Tresser N, Rolf C, Cohen M. Plasma cell granulomas of the brain: Pediatric case presentation and review of the literature. Childs Nerv Syst 1996;12:52-57

9. Lacoste-Collin L, Roux FE, Gomez-Brouchet A, et al. Inflammatory myofibroblastic tumor: A spinal case with aggressive clinical course and ALK expression. J Neurosurg 2003;98:218-21

10. Buccoliero AM, Caldarella A, Santucci M, et al. Plasma cell granulomaan enigmatic lesion: Description of an extensive intracranial case and review of the literature. Arch Pathol Lab Med 2003;127:e220-23

11. Kilinc M, Erturk IO, Uysal H, et al. Multiple plasma cell granuloma of the central nervous system: A unique case with brain and spinal cord involvement: case report and review of literature. Spinal Cord 2002;40:203-6

12. Hsiang J, Moorhouse D, Barba D. Multiple plasma cell granulomas of the central nervous system: case report. Neurosurgery 1994:35:744-47

13. Le Marc’hadour F, Lavieille JP, Guilcher C, et al. Coexistence of plasma cell granulomas of lung and central nervous system. Pathol Res Pract 1995;191:1038-45

14. Brandsma D, Jansen GH, Spliet W, et al. The diagnostic difficulties of meningeal and intracerebral plasma cell granulomas: Presentation of three cases. J Neurol 2003;250:1302-6

15. Park SH, Whang CJ, Sohn M, et al. Idiopathic hypertrophic spinal pachymeningitis: A case report. J Korean Med Sci 2001;16:683- 88

16. Sciot R, Dal Cin P, Fletcher CD, et al. Inflammatory myofibroblastic tumor of bone: Report of two cases with evidence of clonal chromosomal changes. Am J Surg Pathol 1997;1:1166-72

17. Su LD, Atayde-Perez A, Sheldon S. et al. Inflammatory myofibroblastic tumor: Cytogenetic evidence supporting clonal origin. Mod Pathol 1998;11:364-68

18. Griffin CA, Hawkins AL, Dvorak C, et al. Recurrent involvement of 2p23 in inflammatory myofibroblastic tumors. Cancer Res 1999;59:2776-80

19. Biselli R, Boldrini R, Ferlini C, et al. Myofibroblastic tumors: Neoplasias with divergent behavior. Ultrastructural and flow cytometric analysis. Pathol Res Pract 1999;195:619-32

20. Lawrence B, Perez-Atayde A, Hibbard MK, et al. TPM3-ALK and TPM4-ALK oncogenes in inflammatory myofibroblastic tumors. Am J Pathol 2000;157:377-84

21. Bridge JA, Kanamori M, Ma Z, et al. Fusion of the ALK gene to the clathrin heavy chain gene, CLTC, in inflammatory myofibroblastic tumor. Am J Pathol 2001;159:411-15

22. Cools J, Wlodarska I, Somers R, et al. Identification of novel fusion partners of ALK, the anaplastic lymphoma kinase, in anaplastic large cell lymphoma and inflammatory myofibroblastic tumor. Genes Chromosomes Cancer 2002;34:354-62

23. Ma Z, Hill DA, Collins MH, et al. Fusion of ALK to the Ran- binding protein 2 (RANBP2) gene in inflammatory myofibroblastic tumor. Genes Chromosomes Cancer 2003;37:98-105

Yoon Kyung Jeon, MD, PhD, Kee-Hyun Chang, MD, PhD, Yeon-Lim Suh, MD, PhD, Hee Won Jung, MD, PhD, and Sung-Hye Park, MD, PhD

From the Departments of Pathology (YKJ, SHP), Radiology (KHC), and Neurosurgery (HWJ), Seoul National University College of Medicine, Seoul, Korea; and Department of Pathology (YLS), Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.

Send correspondence and reprint requests to: Sung-Hye Park, MD, PhD, Department of Pathology, Seoul National University College of Medicine, 28 Yongon-dong, Chongno-gu, Seoul, 110-799, Republic of Korea; E-mail: [email protected].

Supported by a grant from the Seoul National University College of Medicine.

Copyright American Association of Neuropathologists, Inc. Mar 2005

USDA Shifts Food Pyramid’s Geometry

Apr. 16–WASHINGTON–In trying to replace the famous food pyramid, the U.S. Department of Agriculture has made a bold though risky decision.

When USDA officials unveil their new food guidance program in the next few days, it will include an updated icon to replace the current pyramid, though USDA officials are remaining mum on its design. But whether it’s a pyramid, rectangle or rhombus, the new symbol won’t include a comprehensive diet plan, as the current pyramid does.

Rather, the graphic will be part of a larger Food Guidance System and will include just a few motivational slogans–urging consumers, for instance, to count calories and exercise regularly. The details of the nutritional advice will be explained in printed material and on a revamped, interactive Web site.

“The reason we talk about it as a food guidance system is, no one graphic can carry 23 recommendations,” said Eric Hentges, executive director of the department’s Center for Nutrition Policy and Promotion, who is overseeing the project. “You can’t hang all the necessary information on one graphic. It just won’t work.”

While recognizing the difficulties of reversing American eating habits, several nutrition and marketing experts said the USDA’s upcoming announcement is fraught with peril. If the USDA abandons the pyramid, recognized by 80 percent of Americans, will it lose its connection with the public? And will the public ignore a diet plan that is too complicated to be contained on a refrigerator magnet?

“If you have to go look on the Internet to find details, that’s not going to have much impact on what people consume,” said Walter Willett, a professor of epidemiology and nutrition at Harvard University who devised his own pyramid based on what he believes are healthier foods. “There’s a reality that we have to convey what are the most important issues in a simple way. The key messages can’t be buried in fine print.”

Willard Bishop Jr., a food retail consultant based in Barrington, likened the pyramid to a highly successful brand name that consumers associate with the government trying to help them.

“I don’t think most people understand it, but they know it exists,” Bishop said. “The risk of losing your brand equity is losing your relevance.”

However, Bishop said if the USDA provides a “ladder” to easily bridge the gap between the new graphic icon and the details of the Food Guidance System, “this could be an exceptionally powerful program.”

“The devil will be in what the ladder looks like,” he said.

Agriculture Department officials had similar ambitions in 1992 when they unveiled the food pyramid along with a 32-page booklet of more detailed nutrition advice. But few nutrition professionals or consumers were aware of the booklet, and as a result the public came to depend almost entirely on the food pyramid for federal nutrition advice.

The pyramid is being revamped because it is considered confusing and contains outdated–some say unhealthy– advice. More important, the pyramid’s advice has been largely ignored by the public.

Department officials have revealed few details of how they will motivate the public to move beyond the new graphic symbol and seek out more nutrition information on the Internet or elsewhere.

The USDA is counting on media coverage of its unveiling to help publicize the Food Guidance System, since it has a negligible budget to do so on its own. And Hentges said he also is counting on the food industry to create healthier products that will make it easier for Americans to follow the federal advice.

Despite the secrecy surrounding the Food Guidance System, particularly about the new symbol, there’s little mystery about what the government will recommend for good health.

Its advice will be based on a 71-page booklet, “Dietary Guidelines for Americans 2005,” released in January that resulted from a 1 1/2-year examination of the latest science by a panel of experts.

The guidelines make 23 key recommendations, along with 18 more targeted for specific groups such as the elderly and overweight children. They include eating vastly more fruits, vegetables and whole grains, limiting unhealthy fats, salt and added sugar and exercising for as much as 90 minutes per day to stay fit.

But the dietary guidelines were intended, not for the public, but for educators and nutritionists and for government bureaucrats who oversee federal nutrition programs like school lunches.

The Food Guidance System is supposed to distill those 71 pages of findings into a much more consumer-friendly message.

So while the dietary guidelines provided a blueprint for healthy lifestyles, the Food Guidance System is expected to perform the much harder task of persuading Americans, two-thirds of whom are overweight or obese, to change their behavior.

While remaining cagey on the details, Hentges said the Food Guidance System will include three elements: a graphic to motivate Americans to seek out healthier lifestyles, an educational framework to detail how to do so, and a set of interactive tools to enable individuals to receive personalized health and nutritional data.

Hentges said the problem with the food pyramid is that all of the federal advice was lumped into one symbol. If someone did not understand the content, and many did not, then the food pyramid didn’t work for them.

As an example, he pointed out that while the pyramid recommends 6 to 11 servings of grains, it doesn’t explain that the number of servings isn’t a matter of personal choice; rather, it depends upon the different caloric needs of individuals. In addition, he said many Americans are confused about what a single serving size means.

The new Food Guidance System will provide people seeking a healthier lifestyle a much more specific action plan, officials said. And it should be easier to decipher; for instance, serving sizes will be listed in household measures such as ounces and cups.

Bill Layden, director of food and nutrition practice at the public relations firm Edelman Worldwide, said he agreed that it was unrealistic to expect a simple icon to convey enough information for Americans to maintain a healthy lifestyle. Layden pointed out that oversimplified diet plans–such as no-fat and low-carb diets–have already caused enough trouble.

“There’s no question that the pyramid that we knew did not achieve its objectives,” Layden said, but added, “We don’t expect people to learn how to drive a car by looking at a brochure. We shouldn’t expect people to learn how to eat by looking at a brochure either.”

Layden, who used to direct the nutrition promotion staff at the USDA’s Center for Nutrition Policy and Promotion, said he hopes the department keeps using the pyramid shape to induce Americans to seek out more information about maintaining a healthy lifestyle.

“There are very few brands that have achieved the level of recognition and awareness as the pyramid,” he said, adding, “The idea of perhaps providing a device that will prompt people to learn more could be very successful.”

Marion Nestle, a professor of nutrition at New York University, suggested that while advice on the current pyramid is flawed, the shape itself “has a lot going for it” and could be salvaged with some minor tweaking.

“Personally, I don’t think it’s all that hard to advise people to eat less [watch portion size], move more, and eat lots of fruits, vegetables, and whole grains,” said Nestle, the author of “Food Politics: How the Food Industry Influences Nutrition and Health,” in an e-mail. “But if the USDA did that, they might have to say what to eat less of and therein lies the problem.

“The pyramid needs to be interpreted as saying, ‘eat more from the bottom and less from the top,'” she added. “Less from the top means fewer junk foods–snacks, sodas, candy and the like. The USDA can’t say that, except indirectly, or the makers of those foods would get upset.”

Hentges has repeatedly denied that the food industry has influenced the process.

—–

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USING PORTFOLIOS TO Evaluate Achievement of Population-Based Public Health Nursing Competencies in Baccalaureate Nursing Students

ABSTRACT

Public health nurses from 13 local public health agencies and nurse educators from five schools of nursing developed population- based public health nursing competencies for new graduates and novice public health nurses. Educators in one nursing program used a portfolio assignment to measure achievement of the competencies by traditional and RN to BSN students in a community health nursing course. Data were collected from surveys and focus groups to determine students’ responses to the portfolio and their use of population-based public health nursing concepts. The assignment enhanced students’ critical thinking skills; however, concerns about the structure and evaluation of the portfolio decreased student satisfaction. Recommendations are made for improving the portfolio format, increasing students’ valuing of the portfolio, managing the tension between assessment and learning, and orienting clinical agency staff and nursing instructors.

Keywords Portfolio – Competencies – Evaluation – Population- Based Public Health Nursing – Baccalaureate Nursing Students – Critical Thinking

IN TODAY’S CHALLENGING HEALTH CARE ENVIRONMENT, where more must be done with fewer dollars, nurses must be able to clearly articulate what they do. The development of clearly defined competencies, and the identification of strategies to measure those competencies, is critical to the education of a competent nursing workforce. * THIS STUDY EVALUATED THE EFFECTIVENESS OF USING PORTFOLIOS TO ASSESS THE ACHIEVEMENT OF POPULATION-BASED PUBLIC HEALTH NURSING COMPETENCIES BY BACCALAUREATE NURSING STUDENTS. The competencies were developed for new graduates and novice public health nurses by the Henry Street Consortium, a partnership of five colleges/schools of nursing and 13 local public health agencies (1). The Henry Street Consortium is one of five Local Linking Projects supported by the Public Health Nursing Practice and Education to Promote Population Health Grant, awarded to the Minnesota Department of Health by the Division of Nursing, Health Services and Resources Administration. The grant also provided for training and support of public health nurse preceptors in local agencies (2). Members of the consortium reviewed public health and public health nursing competencies found in the literature and spoke with public health nurses from local agencies before identifying 11 competencies. (The competencies are itemized in Table 4.) The competencies are used in conjunction with the Public Health Intervention Wheel, which specifies three levels of practice (community, systems, and individual/family) and 17 public health interventions (3). (See Figure 1.)

Review of Literature The increasing emphasis on population health requires reframing and updating the knowledge and skills of public health professionals. Today’s public health nurse (PHN) needs knowledge about the measurement of health status, data analysis strategies, organizational change, health economics, and the politics of health. Because PHNs collaborate with other disciplines and communities to solve problems, develop, evaluate, and implement health policy, measure quality of care and health outcomes, and analyze how social determinants affect health (4), they require skills in working with interdisciplinary teams, conducting program evaluation, and building coalitions (5).

Competency-based models provide a structure for identifying and measuring student learning outcomes that are connected to the skills needed for contemporary nursing practice (6,7). Kaiser and Rudolph (8), who evaluated the use of American Nurses Association (ANA) standards for community and public health nursing (9,10) as the basis for a clinical evaluation tool, pointed to the need for additional constructs for the identification of competencies not reflected in ANA standards. Although the standards were found to be useful for organizing the performance evaluation tool, measurement strategies and evaluation tools must accurately reflect expected competencies for practice (11). Planning processes for populations add complexity to public health nursing practice. Thus, the competencies required must reflect the socialization and learning experiences needed for populationbased care.

Figure I

Public Health Intervention Wheel

The literature identifies many benefits derived from the use of portfolios in teaching and in the evaluation of competency achievement. Portfolios have the potential to enhance the achievement of learning outcomes and competencies while providing a structure for assessing the achievement of competencies (12) and documenting the achievement of skills and learning experiences. The portfolio is defined as a “focused purposeful collection of student work that documents evidence of traditional and nontraditional sources of student learning, progress, and achievements over time” (13, p. 209). It results from a collaborative process between students and faculty, with the faculty role that of coach and mentor rather than didactic teacher (13-15).

In an integrative literature review and meta-analysis of the use of portfolios and the assessment of competence, McMullan and colleagues (16) determined that the use of portfolios is based on theories of adult and experiential learning. Portfolios can be an effective strategy to promote lifelong learning and professional goal achievement for nurses at the novice, experienced, and expert levels (17,18).

Self-reflection, whereby one analyzes and synthesizes one’s thoughts and actions (13), is a critical component of educational portfolios. Sorrell and colleagues (19) analyzed nursing students’ reflective essays for critical thinking outcomes. They identified two themes: 1) critical thinking was expressed by “seeing” things related to nursing practice in new ways, and 2) creative thinking was present, along with analysis and synthesis. McMullan and colleagues pointed out that the reflective component of the portfolio encourages integration of ethics and values and helps frame practice in theory (16).

Portfolios in which students reflect on their progress in competency development have been found to promote active student engagement in the learning process (20). Karlowicz (21) suggested that the use of portfolios increases student responsibility for learning, promotes self-confidence as students witness their own development, and enhances faculty-student interactions. For educators, the use of portfolios to evaluate student learning can encourage the examination of the relevance of teaching strategies and assignments to course objectives and program outcomes (21).

Limitations of the portfolio approach must be considered. First, there is a lack of evidence to support the validity of the portfolio score as a measure of the acquisition of knowledge and skills that enable students to provide competent nursing care. second, the reliability of the scoring rubric between faculty members must be tested. Other concerns include the time commitment needed for adequate portfolio development and storage issues (21).

Method A portfolio assignment to measure achievement of the population-based public health nursing competencies was developed and piloted with two groups of baccalaureate students: 43 generic baccalaureate students working in six clinical groups in local public health agencies, and 45 RN to BSN students assigned to preceptors in a variety of community settings. Seven faculty members who taught community health nursing participated.

Students were instructed to complete portfolios with four components: 1) a notebook with sections for each competency; 2) a table of contents; 3) artifacts, which could be assignments, evaluations, teaching plans or outlines, photos, pamphlets on resources or programs, and meeting agendas; and 4) reflections for each competency. Students could use the same artifact for more than one competency and could include several artifacts for one competency if they desired.

Table I. Portfolio Evaluation: Student Survey Responses

An online discussion forum over the duration of the course and a portfolio “help lab” offered several weeks before the portfolio was due provided support to students as they worked on the assignment. Faculty members developed an evaluation rubric consisting of four criteria:

* Artifacts demonstrate the accomplishment of competencies.

* Reflections make connections to population-based public health nursing theory, knowledge, and practice.

* Personal and professional growth through self-examination is demonstrated.

* The portfolio is clearly organized, scholarly, and creative. During the grading process, faculty members met to compare scoring strategies.

Evaluation methods included a survey, focus groups with students, and an analysis of portfolio content. Generic BSN students (n = 35) completed an electronic version of the survey; RN to BSN students (n = 18) completed a paper-and-pencil version. The survey used a four- point Likert scale (4 = strongly agree, 1 = strongly disagree) and open-ended questions to assess students’ overall impressions of the portfolio assignment, how portfolios contributed to the integration of theory intopractice, and their views of the portfolio process.

A graduate student and faculty member who were not involved with students conducted two focus groups with generic students (n = 12) and four focus groups with RN to BSN students (n = 37). The researchers determined mean scores for agreement/disagreement on survey items and used content analysis to identify themes in the focus group responses. In addition, a random sample of nine portfolios submitted by RN to BSN students was analyzed to identify examples of critical thinking that illustrated comprehension of population-based public health nursing. Portfolios for the generic students were returned prior to graduation and were not available for analysis.

Table 2. Focus Group Themes for Traditional Students

The study was approved by the university Institutional Review Board. All students who agreed to participate in the research signed consent forms.

Table 3. Focus Group Themes for RN to BSN Students

Results Table 1 summarizes mean scores on survey items for traditional and RN to BSN students. Most students agreed that portfolios helped explain the role of the PHN and facilitated their use of population-based public health nursing ideas and language. Also, they agreed that clinical experiences provided an adequate number of artifacts or activities to demonstrate the competencies. Students expressed less satisfaction with the portfolio process and indicated that the assignment did not influence them to think about employment as a PHN. RN to BSN students generally disagreed that the portfolio facilitated the learning of new skills.

Responses to open-ended survey questions revealed that the portfolio assignment helped students recognize the number of public health nursing interventions they had accomplished during their clinical experience. Students repeatedly indicated that they had to think critically when recognizing and applying public health nursing interventions. Students also commented that they learned about themselves and the kinds of nursing they would like to practice in the future. Certain concerns were also revealed: 1) the portfolio was not useful if the student was not interested in public health nursing; 2) students felt they had to make up reflections based on what the instructor wanted to hear; 3) it was difficult to understand how to connect the artifact and the competency; and 4) it was difficult to understand how to complete the reflections.

Key themes from the focus group analysis varied for the two groups of students. Four themes emerged for traditional students: 1) confusion, 2) devaluation of the portfolio, 3) critical thinking, and 4) increased understanding of the Public Health Intervention Wheel (Table 2). Students frequently indicated that they were confused about the directions for the project, portfolio components, and expectations for grading. They did not seem to appreciate the reflection process and questioned the worth of the portfolio assignment. However, their comments indicated that they recognized that the portfolio promoted the development of critical thinking skills. Some students commented they had developed a more complete and comprehensive understanding of the Public Health Intervention Wheel.

Eight themes emerged for the RN to BSN students: 1) difficulty finding experiences for the competencies, 2) lack of preceptor understanding of the competencies, 3) confusion, 4) the time- consuming nature of the portfolio, 5) lack of benefit for practicing nurses, 6) expectations for reflective writing, 7) seeing the whole picture, and 8) appreciation for the scope of public health nursing practice (Table 3). Confusion about how to do the portfolio was a theme shared by both groups. RN to BSN students emphasized the intense time commitment involved in completing the portfolio. Some indicated that they had a limited pool of opportunities to choose from, which made it necessary for them to go outside their clinical site to complete the competencies. Students indicated that their preceptors lacked an adequate understanding of the competencies and the Public Health Intervention Wheel.

Table 4.

Examples of Critical Thinking for Population-Based Public Health Nursing Competencies in RN to BSN Student Portfolios (n = 9)

Table 4.

Examples of Critical Thinking for Population-Based Public Health Nursing Competencies in RN to BSN Student Portfolios (n = 9)

Although the RN to BSN students commented that the portfolio assignment was not beneficial for RNs who already had many practice experiences, several indicated that the portfolio helped them see the whole picture of public health nursing and the extent of its influence on the community. Table 4 provides examples from RN to BSN student portfolios of incidents of critical thinking for each public health competency.

Discussion The differences in focus group themes between the traditional and RN to BSN students are likely attributable to the practice experiences and work and family responsibilities generally characteristic of degree-completion (22). Their comments reflected the desire to have their previous practice experiences recognized, which in this study may have contributed to the perception that the portfolio was less beneficial for them.

Data on the lack of understanding of the competencies by preceptors was an important finding. Nursing faculty members for the course realized that RN to BSN students experienced a very different clinical environment – placement with preceptors in schools, clinics, and occupational settings versus placement in official public health agencies with an instructor present. Preceptors for the RN to BSN students generally had little familiarity with the competencies or the Public Health Intervention Wheel.

The response that some students felt they had to “make things up” or write what they thought their instructor wanted to hear is a concern. This response conflicts with the goal of reflective writing, which focuses on the unique experiences and thinking of the writer rather than on an ideal or expected response. Mistakes, negative feelings, and difficulties, when examined, have great potential to promote growth. McMullan and colleagues (16) suggest that the inclination to write what the assessor wants to read may be exacerbated when portfolios are used for summative assessment. Summative assessment of the portfolio may contribute to a dislike of the portfolio, a devaluation of its worth, and a decrease in its efficacy for enhancing learning by stifling honest and examined reflective writing. The challenge is to promote student autonomy in the learning process while using the portfolio as an assessment measure.

Confusion about the portfolio format, connection of the artifact to the competency, and the process of reflective writing can be attributed to two factors. First, the portfolio was piloted for the first time in only one clinical course and was new to both faculty members and students. No examples of previously completed portfolios were available. second, not all community health nursing faculty members had participated in the development of the competencies. The instructors varied in their understanding of the competencies and the assignment.

Finally, data demonstrated that the portfolio assignment succeeds in promoting critical thinking for both traditional and RN to BSN students. Selections from the student portfolios featured in Table 4 show both the variety of experiences and quality of students’ analyses of their actions, interactions, and comprehension of population-based public health nursing. In other words, they “got it.” The portfolio increased student understanding of the role of the PHN and contributed to their accurate application of the Public Health Intervention Wheel to practice. Students gained the conceptual understanding and language needed for population-based nursing practice.

Implications for Portfolio Use in Nursing Education Based on study findings, several changes have been instituted in the implementation of the portfolio assignment. The structure of the assignment has been refined; there is increased emphasis on explaining the value and purpose of the assignment; and clinical agency staff receive an orientation to the assignment and the competencies.

Faculty members have added a page for each competency to the course manual. They encourage students to take notes on the activities and artifacts associated with the competencies to serve as stimuli for in-depth reflective writing.

Timing is seen as crucial to the students’ understanding and acceptance of the assignment. Faculty members introduce the portfolio assignment after the first week of the semester when students are generally less overwhelmed with the many details of course requirements. Portfolio examples are placed on reserve in the library. To encourage consistency and clarity of instructions and grading criteria among faculty, team members are required to participate in a workshop conducted by a portfolio expert. In addition, instructors are intentional about helping RN to BSN students link their clinical experiences with competencies.

Staff at the participating clinical agencies are expected to have knowledge of the competencies, artifacts, instructions, and grading rubric for the portfolio as well as the Public Health Intervention Wheel. Those who have a deficit in these areas are offered opportunities to help increase their knowledge.

Several strategies have been implemented to help students understand the value of the portfolio assignment. The portfolio now accounts for a greater percentage of the course grade to reflect the time investment. Instructors emphasize the use of portfolios for professional development, writing one’s resume, or preparing for a job interview. In addition, a celebration is held to share learning among peers and instructors in a collegial manner, and the nursing department is planning to use portfol\ios to document student learning for the entire curriculum. Instructors attempt to clarify the dual purposes of the portfolio – learning and assessment. They incorporate strategies to encourage and reinforce reflective writing that help students examine their mistakes and negative feelings. An environment of acceptance is enhanced by having students submit their portfolios twice for ungraded feedback before the entire portfolio is due. It is hoped that these strategies will promote student honesty in writing about their learning and growth as public health nurses. (See Figure 2 for the revised portfolio assignment.)

Figure 2. Portfolio: Population-Based Public Health Nursing Competencies

Conclusion The portfolio assignment enhanced students’ critical thinking ability as demonstrated in their application of population- based public health nursing concepts. A comment on the course evaluation form by an RN to BSN student illustrates that the purpose of the portfolio was realized: “The clinical experience and relating the portfolio to the intervention wheel was very helpful for organizing my learning and doing self-evaluation of my learning.” While portfolios have the potential to promote the development of critical thinking, nurse educators also need to consider how using the portfolio for assessment will affect students’ reflections about their learning.

The public health nursing competencies achieved by the students will serve as a strong foundation for graduates who choose public health nursing lor their practice arena. The portfolio approach promotes self-evaluation and reflective practice. Nurses who develop competence through reflecting about their actions will be better able to anticipate and respond to the challenges of an ever- changing health care environment.

Data demonstrated that the portfolio assignment SUCCEEDS in promoting critical thinking for both traditional and RN to BSN students. Selections from the student portfolios show both the variety of experiences and quality of students’ analyses of their actions, interactions, and comprehension of population-based public health nursing. In other words, they “got it.”

References

1. Center for Public Health Nursing, Minnesota Department of Health. (2003). Entry level population-based public health nursing competencies for the new graduate or novice public health nurse. [On- line]. Available: http://www.health.state.mn.us/divs/chs/phn/ henrystreet16.pdf.

2. Center for Public Health Nursing, Minnesota Department of Health. (2003). Linking public health nursing practice and education to promote population heath: Preparing public health nurses for population-based practice. [On-line]. Available: http:// www.health.state.mn.us/divs/chs/phn/partnerships.html.

3. Keller, L. O., Strohschein, S., Lia-Hoagberg, B., & Schaffer, M. A. (2004). Population-based public health interventions: Practice- based and evidence-supported (Part I). Public Health Nursing, 21(5), 469-487.

4. Berkowitz, B. (2002). Preserving our mission. Public Health Nursing, 19(5), 319-320.

5. Gebbie, K. M., & Hwang, I. (2000). Preparing currently employed public health nurses for changes in the health system. American Journal of Public Health, 90(5), 716-721.

6. Lenburg, C. B. (1999). The framework, concepts, and methods of the competency outcomes and performance assessment (COPA) model. Online Journal of Issues in Nursing. Available: http:// www.nursingworld.org/ojin/topic10/tpc10_2.htm

7. Luttrell, M. F., Lenburg, C. B., Scherubel, J. C., Jacob, S. R., & Koch, R. W. (1999). Competency outcomes for learning and performance assessment: Redesigning a BSN curriculum. Nursing and Health Care Perspectives, 20(3), 134-141.

8. Kaiser, K. L., & Rudolph, E. J. (2003). Achieving clarity in evaluation of community/public health nurse generalist competencies through development of a clinical performance tool. Public Health Nursing, 20(3), 216-227.

9. American Nurses Association. (1986). Standards of community health nursing practice. Kansas City, MO: Author.

10. American Nurses Association. (1999). Scope and standards of public health nursing practice. Washington, DC: Author.

11. Hawranik, P. (2000). The development and testing of a community health nursing clinical evaluation tool. Journal of Nursing Education, 39(6), 266-273.

12. Day, M. (1998). Community education: A portfolio approach. Nursing Standard, 13(10), 40-44.

13. Wenzel, L. S., Briggs, K. L., & Puryear, B. L. (1998). Portfolio: Authentic assessment in the age of the curriculum revolution. Journal of Nursing Education, 37(5), 208-212.

14. Jasper, M. (1995). The potential of the professional portfolio for nursing. Journal of Clinical Nursing, 4(4), 249-255.

15. Ramey, S. L., & Hay, M. L. (2003). Using electronic portfolios to measure student achievement and assess curricular integrity. Nurse Educator, 28(1), 31-36.

16. McMullan, M., Endacott, R., Gray, M. A., Jasper, M., Miller, C. M. L., Scholes, J., & Webb, C. (2003). Portfolios and assessment of competence: A review of the literature, journal of Advanced Nursing, 41(3), 283-294.

17. Bell, S. K. (2001). Professional nurse’s portfolio. Nursing Administration Quarterly, 25(2), 69-73.

18. Kelly, J. (1995). The really useful guide to portfolios and profiles. Nursing Standard, 9(32), 3-12.

19. Sorrell, J. M., Brown, H. N., Silva, M. C., & Kohlenberg, E. M. (1997). Use of writing portfolios for interdisciplinary assessment of critical thinking outcomes of nursing students. Nursing Forum, 32(4), 12-25).

20. Tracy, S., Marino, G. J., Richo, K. M., & Daly, E. M. (2000). The clinical achievement portfolio: An outcomes-based assessment project in nursing education. Nurse Educator, 25(5), 241-246.

21. Karlowicz, K. A. (2000). The value of student portfolios to evaluate undergraduate nursing programs. Nurse Educator, 25(2), 82- 87.

22. Sedlak, C. A. (1999). Differences in critical thinking of nontraditional and traditional nursing students. Nurse Educator, 24(6), 38-44.

About the Authors Marjorie A. Schaffer, PhD, RN, is a professor at Bethel University; St. Paul, Minnesota, where Pamela Nelson, MS, RN, is an assistant professor. Emily Litt, MS, RN, PHN, is a planner with the Minnesota Department of Health.

The authors wish to thank the Center for Public Health Nursing at the Minnesota Department of Health for partial funding through their Linking Public Health Nursing Practice and Education to Promote Population Health grant (#1D11HP00330-01) from the Division of Nursing, Bureau of Health Professions, Health Resources and Services Administration. For more information, contact Dr, Schaffer at m- [email protected].

Copyright National League for Nursing, Inc. Mar/Apr 2005

Unm Program Eases Access to Treatment of Hepatitis C

Orlando Velarde is one of 32,000 or more New Mexicans infected with a liver disease called hepatitis C. He learned he had it in 1999, while hospitalized for a blood clot in his arm.

He probably had been sick for two decades. But like many people with hepatitis C, he had no symptoms. Velarde believes he caught the virus through a blood transfusion in 1980 — long before plasma banks screened for the disease.

Hepatitis C is a leading health concern in New Mexico, where deaths from liver disease and cirrhosis outpace the nation. (Alcoholism contributes to the problem, too.)

Less than 6 percent of New Mexicans with hepatitis C are getting treated. Part of the problem is access.

Velarde understands. To get the regimen of shots and pills, which can last a year, he had to drive from his home in Espanola to Santa Fe. There were no other options at the time.

The side effects alone can be grueling — fatigue, nausea, pain. Adding a one-hour round-trip drive to that can make it agonizing.

Velarde dropped out of the program. But now he’s back on track, because Health Centers of Northern New Mexico in Espanola began offering the treatment last year.

“I am now at seven months and feel good about getting cured,” said Velarde, in his mid-40s.

Mainstream medicine offers one treatment for hepatitis C. It cures 50 to

80 percent of cases.

With a three-year grant from the federal government, The University of New Mexico School of Medicine has made treatment more available. Project ECHO, developed by Dr. Sanjeev Arora, educates primary-care physicians on the treatment of hepatitis C and provides weekly support, via telephone, with a broad-based group of specialists at UNM.

Under this telemedicine program, new outlets for care in Northern New Mexico include: Health Centers of Northern New Mexico, Pojoaque Primary Care, Santa Fe Indian Hospital and New Mexico State Penitentiary in Santa Fe. Other locations are: Bernalillo, Albuquerque, Las Cruces and five prisons.

At an open house Thursday, Health Centers of Northern New Mexico will spread the word that treatment is available. “Although not everyone is cured, it helps the liver even if you aren’t cured,” Bjeletich said.

The treatment isn’t free. For uninsured patients, the clinic can bring the cost down to $1,000, using donated services and medicines, she said. Otherwise, insurance usually covers the cost of treatment, minus copays.

Espanola’s first patient — Velarde — started in August 2004. Now the clinic has six. Patients must be clean from drugs and sober before beginning treatment, Bjeletich said.

The course of therapy lasts six months to a year, depending on what strain of the virus the patient has. Besides taking pills (ribavirin) daily, the patient must self-inject once a week with interferon. Like chemotherapy, the treatment kills infection- fighting blood cells, making patients vulnerable to germs.

New Mexico has lots of deaths related to hepatitis C, according to Arora, though firm numbers aren’t available. The disease can lead to cancer of the liver, cirrhosis of the liver and death. For people with significant liver disease, treatment is urgent, he said.

Without medical care, one-fourth of hepatitis C patients develop cirrhosis of the liver — a condition of irreversible scarring — within 20 to 30 years of infection, Arora said.

Plenty of others, however, never suffer enough liver damage to endanger their lives. For them, treatment is optional, Arora said. They must decide whether they’d rather live with the fatigue, sleep disturbances, depression and loss of sexual vitality that hepatitis C can cause or endure the side effects of treatment, he said.

Hepatitis C is commonly transmitted through contaminated needles or straws during heroin or cocaine use. But nearly 10 percent of New Mexicans with the virus contracted it through blood transfusion — a risk that has virtually disappeared, Arora said. Tattoos, especially those made in prison, can be worrisome; the virus can live up to a week in ink.

“There’s a stigma attached to hepatitis C that does not need to be there,” Bjeletich said. “There’s lots of ways to get it.”

And to date, there’s no vaccine against it.

(Sidebar)

Hepatitis C: The silent epidemic

Most people infected with hepatitis C have no symptoms. But this disease of the liver can kill you.

In New Mexico, hepatitis C is

20 times more prevalent than AIDS, according to University of New Mexico School of Medicine

Dr. Sanjeev Arora.

The virus is transmitted by blood-to-blood contact. To find out who is at risk and should take a blood test, Health Centers of Northern New Mexico in Espanola developed a screening tool:

1. Have you ever been diagnosed with hepatitis C? (If yes, stop here.)

2. Have you ever injected yourself with illegal drugs, even one time many years ago?

3. Do you have HIV or AIDS?

4. Did you have a blood transfusion or organ transplant before July 1992?

5. Did you have dialysis or other blood products before July 1992?

6. Are you a chronic hepatitis B carrier?

7. Have you ever snorted cocaine, even once?

8. Do you have tattoos or body piercings?

9. Have you been in jail or prison?

10. Have you had sex with someone who is hepatitis C positive?

11. Have you shared razors, toothbrushes or had blood contact with someone who is hepatitis C positive?

12. Do you drink more than four drinks a day, or have you ever been diagnosed as alcoholic?

The treatment program — six months to one year of shots and pills — is not right for everyone. Health Centers of Northern New Mexico offers information to help patients decide if they want treatment.

The New Mexican

If you go …

What: Hepatitis C Awareness Day, including free lunch, risk screenings and information.

When: 11:30 a.m. to 1:30 p.m. Thursday.

Where: Health Centers of Northern New Mexico, 620 Coronado St., Espanola.

Who: Community leaders, relatives of high-risk people, people with hepatitis C.

Follow-up: By appointment only. The Espanola clinic will offer blood testing May 4 to anyone considered at high risk for the disease.

Information: Barbara Bjeletich, 753-7395

`Doctors Agreed to Fomema’s Procedures’

KUALA LUMPUR: All doctors on Fomema Sdn Bhd’s panel have signed a contract to conform to the standard operating procedures (SOP) laid out by the company.

Fomema general manager Tang Yow Lee, in a statement to The Malay Mail, said the agreement stipulated that doctors must adhere strictly to the SOP in carrying out medical examination on foreign workers under Fomema’s health screening programme.

“We recently issued a revised and updated SOP as part of the new management’s initiative to enhance the monitoring and surveillance system in ensuring quality and integrity of the medical examinations,” he said.

He clarified that the updated SOP was discussed and agreed upon by the authorities, including the Malaysian Medical Association (MMA).

“The doctors also agreed to the SOP, which states that failure to follow or breach any part of the procedures may result in action taken by Fomema, including suspension,” he said, in response to The Malay Mail report on April 9.

It was reported that doctors suspended by Fomema had claimed that the procedures set by the company were not practical.

The doctors had complained to MMA president Datuk Dr N. Arumugam who revealed complaints about the impracticality of certain procedures.

Dr Arumugam noted one `unreasonable’ procedure as doctors being required to determine whether the passports of the workers are genuine.

But Tang refuted that the procedures do not state that.

“Instead, Clause 1.1 of the SOP requires the attending doctor to verify the identity of the foreign worker through his or her passport and that medical examination should not be conducted if the identity cannot be ascertained.

“This procedure is critical as we have detected attempts by healthy persons standing in place of unhealthy foreign workers in order to obtain favourable medical results to extend their work permits,” said Tang.

Tang added that if the identity of the foreign workers is not properly verified at the medical examination, it will affect the integrity of the medical results by the doctors.

Dr Arumugam had also said that Fomema also required the doctors to spend at least 15 minutes to examine the workers.

He had argued that a strict time frame was not necessary as the doctors are professionals and know their responsibilities.

To this, Tang clarified that the time frame is only a guide and that the Health Ministry, MMA and Fomema had agreed that doctors should not carry out too many medical examinations on foreign workers in a day to ensure the quality of the tests is not compromised.

Tang also said Fomema uses the guide as it has detected doctors who have claimed to carry out 100 medical examinations in a day.

The Malay Mail had, on April 7, reported that 25 doctors on Fomema’s panel had been suspended after they were found to have breached procedures including giving misleading information on the workers.

Several doctors were caught allowing a healthy foreign worker to stand in for a sick worker for an X-ray.

The errant doctors were those from the Klang Valley, Johor and Negri Sembilan.

Fomema was given a concession by the Government to carry out medical examinations on foreign workers in 1997. It has 3,300 doctors on its panel.

Mannford Center Seeks to Draw Bigger Crowd

Mannford’s Activity Center has proven popular, but city leaders and board members would still like to see it used by more people.

Two months after the activity center opened its doors in March 2004, its roster showed more than 300 members and has about the same number now.

“We’ve had a lot of positive feedback,” said Cecilia Ward, finance director for the city of Mannford.

“It’s definitely not a money maker and we hope to break even in the future.”

January was the busiest month with lots of people wanting to exercise after making New Year’s resolutions.

Ward said they hope to market the facility to outlying communities and to more Mannford residents.

“Some people still don’t know about it. Hopefully they’ll walk around, notice the facility and come look and see what we have to offer,” she said.

About $2.5 million was allocated for the activity center from a $5.5 million bond issue passed in 2001.

“The community wanted a facility for youth but it’s turned out be a facility for the whole community. We have quite a few senior citizens who participate in water aerobics,” Ward said.

The 12,000-square-foot center at 100 Cimarron St. includes a heated pool, a multipurpose room and a fitness center.

Water aerobic classes tend to be the most popular at the center with 25 people enrolled in the morning and 15 to 20 in the evening.

Center Administrative Assistant Donna Bradfield said the people they have spoken with are happy with the programs offered.

A survey was sent to members and the community requested more free weights, Bradfield said.

As a result, the visitor’s information area is being moved to the lobby and more free weights will be available in the fitness equipment room.

“We’re hoping to get more male members with the free weights. We’re happy to offer that for them,” Ward said.

During spring break, a two-day Sponge Bob mini-camp was held for kids.

“It was a trial program and hopefully we’ll offer more in the future,” Ward said.

Karate classes are available along with swim lessons for youth.

The activity center is open from 6 a.m. to 9 p.m., Tuesday through Friday, 8 a.m. to 8 p.m. Saturday and 1 to 5 p.m. Sunday. The facility is closed Mondays.

The center is open to members only, but a daily swimming pass may be purchased by anyone.

Family memberships cost $30 to $35 a month, and individual memberships cost $20 to $25.

For more information, call 865-6891.

Common Causes of Skin Atrophy

Solar damage

There are many non-malignant changes in the skin that are caused by ultraviolet light, for example mottling, wrinkling, coarseness, telangiectasia and atrophy of the skin in later life. This woman had dry, mottled atrophie skin with multiple solar keratoses on her legs and feet having spent many years living in the Far East and Florida. She was advised to avoid further sun exposure, to apply plenty of emollients and to try 5-fluorouracil cream for the solar keratoses.

Radiodermatitis

Basal cell and squamous carcinomata respond well to radiotherapy but the irradiated skin will develop signs of atrophy afterwards. The skin shown here is scarred, atrophie and has developed telangiectasia over it. In the long term, the area may also develop further basal cell carcinomas at the site. It is therefore advisable to restrict the use of radiotherapy for these lesions to patients who are very elderly and nervous, for large lesions, and for those that may have recurred after surgery.

Lichen sclerosis et atrophicus

The cause of lichen sclerosis et atrophicus is unknown. It may occur at any age. The lesions are shiny, white, smooth-surfaced papules that coalesce symmetrically and become atrophie with telangiectasia and purpuric changes. These skin changes frequently occur around the vulva and anus where patients complain of itching and burning. In men, the foreskin and glans penis may be involved. These problems may lead to shrinkage of the vulval tissue and balanitis xerotica obliterans. Similar skin changes do occur elsewhere on the body as in this patient, who has a typical ‘cigarette paper’-like area on the forearm.

Chronic cutaneous lupus erythematosus

Chronic cutaneous lupus erythematosus or discoid LE presents with a rash that is precipitated by sunlight and appears mainly on the light exposed areas of the face, scalp and limbs. Discoid LE is more common in women. Its cause is unknown. The patient presents with raised red or purpuric plaques with scaling, follicular plugging and hyperkeratosis. Healing starts centrally leaving atrophie scars. Hair follicles on the scalp are destroyed, causing permanent alopecia that cannot be treated. The diagnosis of discoid LE can be confirmed by skin biopsy.

Striae

Striae often cause embarrassment and distress to the patient. It is only occasionally that they may tear or ulcerate. They are particularly common on the breasts and abdomen in pregnant women, as in this case. They are also frequently seen in adolescents during their growth spurt and may also occur in patients on prolonged systemic or potent topical steroids. In Cushing’s disease the striae tend to be wider, deeper and have a greater intensity of colour. Some patients have been helped with topical tretinoin (not to be used in pregnancy), 15-20 per cent trichloracetic peels, and pulsed dye laser treatment.

Disseminated superficial actinic porokeratosis

Disseminated superficial actinic porokeratosis usually develops in patients aged 30-50 years and in those that have enjoyed much exposure to ultraviolet light. It is more common in women and presents with multiple, light-brown, atrophie patches on the extensor surfaces of the limbs and sometimes the face. Occasionally they may itch but are often asymptomatic. There is a risk of developing basal and squamous cell carcinoma. The patient should avoid further sun exposure and protect themselves, and be alert to the signs of any possible malignant change. Topical 5-fluorouracil may induce remission.

Necrobiosis lipoidica

Necrobiosis lipoidica occurs in 0.3 per cent of diabetics. It presents most commonly in women in their thirties but may occur at any age. Asymptomatic, shiny red-brown patches gradually increase in size and become yellow and atrophie. They may be multiple and bilateral. Trauma may encourage the development of ulcers that are painful and which, if they heal, leave scars. It usually occurs on the pretibial area but may also be seen on the face, scalp, trunk and upper limbs. Topical or intralesional steroids may reduce the inflammation of early, active borders of lesions but may in the long run induce further atrophy. Aspirin and dipyridamole can be tried.

Morphoeic basal cell carcinoma

Morphoeic basal cell carcinoma can be difficult to diagnose. This sclerosing basal cell carcinoma may look like a patch of atrophie skin or an old scar. However, it does slowly increase in size and when stretched, the edges of the lesion can be made to stand out. In fact, the lesion is usually much biggerthan it appears at first sight. The lesion is best excised. It is obviously important to remove the whole of the lesion and a wide margin should be allowed if a recurrence is to be avoided.

Copyright Haymarket Business Publications Ltd. Mar 11, 2005

Lymphoma Mimicking a Thyroglossal Duct Cyst in an Adolescent

Abstract

Mass lesions of the head and neck in infants and children can be either developmental, inflammatory or neoplastic. Lymphomas (Hodgkin’s or Non-Hodgkin’s) commonly present as neck lumps in children. Although malignancy is not the commonest aetiology of paediatric cervical lumps, a high index of suspicion is critical to facilitate early diagnosis and treatment of cervical lesions. We present the case of a 15-year-old boy who presented with a solitary midline cervical lesion, which simulated a thyroglossal cyst on clinical examination. However, histopathological examination revealed it to he a Hodgkin’s lymphoma. Related literature is also reviewed.

Key words: Adolescent; Hodgkin’s Disease; Neck; Thyroglossal Cyst

Introduction

The cervical region is a common site for a number of mass lesions, most of which are, in children, of developmental or inflammatory origin. Although malignancy is not the most common aetiology, cervical lumps in infants and children must be considered possibly malignant until proven otherwise.

With the exception of rhabdomyosarcomas of the nasal passages and orbit and, rarely, neuroblastomas that present in the cervical region, malignant lesions tend to present later in childhood or in the teenage years. Congenital and inflammatory lesions, on the other hand, present in infancy and early childhood.

An accurate history and physical examination combined with other diagnostic modalities, such as biopsy or an imaging scan, will usually establish the diagnosis.

Neoplasms involving the head and neck account for 5 per cent of all malignancies occurring in children.1 More than 50 per cent are lymphomas (Hodgkin’s lymphoma (HL) or non-Hodgkin’s lymphoma (NHL)) or soft tissue sarcomas, primarily rhabdomyosarcomas.1 The American Cancer Society estimates that in 2002 there were about 7000 new cases of Hodgkin’s disease in the USA.2

The most common presentation of HL is a painless, firm, fixed cervical mass. The commonest site for presentation is the neck, although deposits may be also found in the mediastinum, liver and bone marrow.

Case report

A 15-year-old boy presented to our department with nine months history of a swelling in the neck. Initial blood tests requested by his general practitioner were normal. Examination of the lump revealed a 1.5-cm, round, non-tender lesion in the midline at the level of the hyoid bone with a firm consistency. The lump was mobile on protrusion of the tongue and swallowing. There were no other lumps in the neck and the rest of the examination was unremarkable. The past medical history was also unremarkable.

Ultrasound examination of the neck revealed a 1.6-cm diameter, ovoid soft tissue mass, superficial to the lower mylohyoid, lying just superior to the hyoid bone. The fact that the mass was lying superficial to the mylohyoid and did not penetrate it did not favour the diagnosis of a thyroglossal cyst. Fine needle aspiration was then carried out. Mucoid material was aspirated.

Histopathological examination of the specimen revealed only blood and lymphoid cells consistent with origin from a lymph node.

Following this, the patient underwent a Sistrunk operation. However, on dissection, there was no evidence of a tract-connection between the lesion and the tongue base; therefore, no dissection of the hyoid bone was carried out. The lesion was sent for histopathological examination, which showed a lymph node in which there was marked expansion of the interfollicular zone and effacement of the architecture (See Figure 1). There was an infiltrate of large atypical cells within the lymph node, some of which were multilobulated, whilst others resembled Reed-Stenberg cells (See Figure 2). These histological appearances were consistent with lymphocyte predominant HL.

Computed tomography (CT) scan of the thorax, abdomen and pelvis did not show any pathology. The patient was subsequently treated with chemotherapy.

Discussion

Cervical lesions are commonly encountered in the paediatric population. In children, in contrast to adults, most cervical lesions are benign. There are three basic classifications: congenital, inflammatory and neoplastic lesions. On a review of 445 cases, 55 per cent of all cases were found to he of congenital origin, 27 per cent of inflammatory origin, and 14 per cent due to neoplasia.3

FIG. 1

A lymph node in which the normal follicular architecture has been effaced (H&E, 20).

FIG. 2

A typical Reed-Stenberg cell of Hodgkin’s disease in a background of small lymphocytes (H&E, 400).

Congenital lesions are common and may include cystic hygromas, haemangiomas, branchial cleft anomalies, thyroglossal duct cysts, and dermoid and epidermoid cysts. In their series review of 102 cases Nicollas et al. reported an incidence of 53.4 per cent for thyroglossal cysts and 11 per cent for dermoid cysts, as the most common midline cervical abnormalities.4 Inflammatory lesions are also commonly encountered and mainly involve lymph nodes. They can be due to a bacterial or a viral cause or, less frequently, to an atypical mycobacterial infection. Malignant lesions include lymphomas, thyroid carcinoma, rhabdomyosarcoma and neuroblastoma.

The differential diagnosis of a thyroglossal duct cyst includes ectopic thyroid tissue, thyroid neoplasms, dermoid or sebaceous cyst, lipoma and submental lymphadenitis. Malignant transformation or malignancy within the cyst is a clinical entity well described in the literature. It is either of thyroid or squamous cell origin, with thyroid papillary carcinoma appearing as the most common form (80 per cent of the cases).5 Ectopic thyroid tissue is identified within the cysts in about 25 to 35 per cent of all cases.1

Dermoid cysts may appear in the midline of the neck and therefore be confused with a thyroglossal duct cyst.6 Other, less common cervical lesions which should be included in the differential diagnosis are pyriform sinus lesions, cervical teratomas, lung herniation, venous aneurysms, torticollis, ranula and midline cervical cleft.

Thyroglossal duct cysts are ectodermal remnants which develop along the line of descent of the thyroid gland from the base of the tongue to the pyramidal lobe of the thyroid gland. They are the most common congenital neck masses. Thyroglossal cysts are more commonly encountered in children between two and 10 years of age, with equal prevalence in both sexes.1 Classically, they are located in the midline at, or immediately adjacent to, the hyoid bone. These cysts are usually soft, smooth and non-tender, and, owing to their attachments to the foramen caecum and hyoid bone, they may rise in the neck when the child swallows or protrudes its tongue, although this is not a consistent finding.

When considering preoperative imaging there does not appear to be a consensus about a single most useful diagnostic modality. Ultrasonography appears as the study of choice for the diagnosis of midline masses, with 10 per cent sensitivity and 95 per cent specificity for detecting thyroid gland tissue.7 It is also of value in the differential diagnosis of masses in the anterior neck region, being especially useful for distinguishing between a thyroglossal duct cyst, a fluid-filled laryngocele and a branchiogenic cyst.8 In the UK the commonest preoperative imaging investigations requested are the ultrasound scan and the radioisotope scan.9 The possibility of the patient’s only functioning thyroid tissue being removed due to being mistaken for a thyroglossal cyst may thus be avoided.

Thyroglossal duct cysts are best treated by complete excision of the cyst, following its sinus tract up to the base of the tongue and including removal of the central portion of the hyoid bone, as described by Sistrunk in 1920.10

Neoplasms of the head and neck region account for 5 per cent of all malignancies occurring in children.1 More than half of these are lymphomas (HL or NHL) or soft tissue sarcomas.1 A malignant lymphoma is a neoplastic transformation of cells that reside in lymphoid tissue. The two main variants are NHL and HL. Both forms typically appear with painless adenopathy, and thus frequently present to the head and neck surgeon.

The type of malignancy encountered differs with age. In adolescents HL is more common.1

Although fine needle aspiration (FNA) of head and neck masses is used routinely in adults, it is not used commonly in children. However, Ramadan et al. showed that FNA in children is a well tolerated method. No complication was noted, and no false negative or false positive results were encountered. In one patient FNA was nondiagnostic and the diagnosis was established after surgical biopsy.11 On comparing FNA with diagnostic open biopsy, FNA reduces the need for more invasive and costly procedures, with potential complications. However, FNA has limitations, as in cases of nonhomogeneous masses or in cases where the histological architecture of the sample needs to be preserved.12

Formal excisional biopsy is preferred over needle biopsy in rapidly enlarging masses, in the presence of systemic symptoms, and when repeated FNA cytology is nondiagnostic. Excisional biopsy ensures that adequate tissue is obtained, both for light microscopy and for appropriate immunocytochemical studies, culture and cytogenetic analysis.

In reviewing the literature, there has been only one other published case \of HL simulating a thyroglossal cyst in a paediatric patient. This was a case of a 14-year-old boy with Robin sequence who presented initially for evaluation of an enlarging lump beneath his chin. As the clinical impression was that of a thyroglossal cyst, surgical excision of the lump was performed and histopathological examination revealed HL of lymphocyte predominance type. However, in this case the lymphoma was more extensive, with deposits in femoral and submandibular lymph nodes. The patient was treated with involved field irradiation and combination chemotherapy with ABVD and MOPP.13

In our case we demonstrated that although ultrasound scanning remains the most commonly used clinical investigation, definitive diagnosis of the cervical lesion was established only after an excisional biopsy. Although the lesion demonstrated all the cardinal clinical features of a thyroglossal duct cyst, the final diagnosis was that of HL. Therefore, emphasis should be placed by an examining physician on early referral for specialist opinion, despite initial clinical examination indicating a benign lesion.

Conclusion

Although malignancy is not the commonest aetiology of paediatric cervical lumps, a high index of suspicion is critical to facilitate early diagnosis and treatment of cervical lesions. The role of the ENT surgeon remains central in the management of head and neck masses and should be the first point of referral by the general practitioner. A thorough ear, nose and throat examination is mandatory. Ultrasonography can be a useful adjunct in evaluating lesions of the head and neck.

Each lesion has to be evaluated by FNA and excision biopsy under a general anaesthetic. Hodgkin’s disease is a rare malignancy, accounting for one in four of all lymphoma cases. A multidisciplinary approach is needed for the management of the paediatric patient with Hodgkin’s disease, involving the ENT surgeon, a paediatrician, an oncologist, and a radiologist.

Acknowledgement

We would like to thank Dr N Williams, Consultant Pathologist, Singleton Hospital, Swansea, UK, for his contribution of the photomicrographs.

* Midline neck swelling in adolescents is usually due to a thyroglossal cyst

* In this case a midline neck swelling was the presentation of Hodgkin’s lymphoma, diagnosed by histological examination of the resected lesion

* Ultrasonography appears to be the imaging modality of choice with midline neck swelling

References

1 Brown RL, Azizkhan RG. Pediatric head and neck lesions. Pediatr Clin North Am 1998;45:889-905

2 American Cancer Society. Hodgkin’s disease. http:// www.cancer.org [5 February 2003]

3 Torsiglieri AJ Jr, Tom LW, Ross AJ 3rd, Wetmore RF, Handler SD, Potsic WP. Pediatric neck masses: guidelines for evaluation. Int J Pediatr Otorhinolaryngol 1988;16:199-210

4 Nicollas R. Guelfucci B, Roman S, Triglia JM. Congenital cysts and fistulas of the neck. Int J Pediatr Otorhinolaryngol 2000;29:55:117-24

5 Motamed M, McGlashan JA. Thyroglossal duct carcinoma. Curr Opin Otolaryngol Head Neck Surg 2004;12:106-9

6 Bloom D, Carvalho D, Edmonds J, Magit A. Neonatal dermoid cyst of the floor of the mouth extending to the midline neck. Arch Otolaryngol Head Neck Surg 2002;128:68-70

7 Gupta P, Maddalozzo J. Preoperative sonography in presumed thyroglossal duct cysts. Arch Otolaryngol Head Neck Surg 2001;127:200-2

8 Baatenburgh de Jong RJ, Rongen RJ. Guidelines for the use of ultrasound in the head and neck. Otorhinolaryngology 1993;55:309-12

9 Brewis C. Mahadevan M, Bailey CM, Drake DP. Investigation and treatment of thyroglossal cysts in children. J Roy Soc Med 2000;93:18-21

10 Sistrunk WE. The surgical treatment of the thyroglossal tract. Ann Surg 1920;71:121-4

11 Ramadan HH, Wax MK, Boyd CB. Fine-needle aspiration of head and neck masses in children. Am J Otolaryngol 1997;18:400-4

12 Liu ES, Bernstein JM, Sculerati N, Wu HC. Fine needle aspiration biopsy of Pediatric head and neck masses. Int J Pediatr Otorhinolaryngol 2001 20;60:135-40

13 Good GM, Isaacson G. Hodgkin’s disease simulating a Pediatric thyroglossal duct cyst. Am J Otolaryngol 2000;21:277-80

ANDREW TARNARIS, MRCS, WIJAYASINGAM GIRIDHARAN, FRCS, DAVID W AIRD, FRCS

From the Department of ENT Surgery, Singleton Hospital, Swansea, Wales, UK.

Accepted for publication: 11 October 2004.

Address for correspondence:

Dr Andrew Tarnaris,

3 Sawel Terrace,

Hendy,

Carmarthenshire SA4 0UY,

Wales, UK.

Fax: +44 (0)1792 884702

E-mail: [email protected]

Dr A Tarnaris takes responsibility for the integrity of the content of the paper.

Competing interests: None declared

Copyright Royal Society of Medicine Press Ltd. Mar 2005

St. George’s Puts Community in the Swim — New Aquatic Center Hosts Teams, Lesson Programs

The Compton Aquatic Center at St. George’s Independent High School in Collierville is open. While the facility is home to the St. George’s swim team, the 28,000-square-foot center will also benefit the entire Collierville, Germantown and Cordova communities.

The 50-meter long, 25-yard wide lap pool is hosting swim lesson programs, swim team opportunities, masters swimming classes, and triathlon training for both children and adults. The facility’s second pool, a smaller 25-yard, four-lane “lesson pool” is home to the Pool School, a lesson program for children age 3 and older.

After a generous donation of $1.7 million from Robert Janice Compton, whose children attend St. George’s, the pool’s official opening was scheduled for fall 2003, but unexpected delays kept swimmers out of the water. Now, 18 months later, the facility is open for use and can easily accommodate up to 250 swimmers at once.

“We hope to raise awareness of, and participation in, competitive swimming events in our community, including all of Collierville, Germantown and Cordova,” said Cory Horton, aquatic director of the center and head coach for the St. George’s junior high and high school swim teams.

Swim meets and other events will be held year-round at the Compton Aquatic Center due to the unique, state-of-the-art building made by Universal Fabric Structures, which allows heating and air circulation while protecting the building’s interior from the elements. Because of its dimensions, the 50-meter pool can be set up for long-course and short-course training. Area high school swim teams rent lanes for practice, and the pool is also home to the Memphis Thunder Aquatic Club swim team, owned by Horton.

“This is the largest indoor aquatic facility in West Tennessee, and I just appreciate the opportunity to work in such a comfortable, modern facility,” Horton said.

In the future, the school plans to host a health fair on the property, complete with a pool party for all the residents of the surrounding communities to enjoy the facility.

Terie L. Box is the public relations director for Memphis Thunder Aquatic Club.

Task-Oriented Versus Emotion-Oriented Coping Strategies: the Case of College Students

The study examined the relationships between stress and coping strategies among 283 college students. Participants completed questionnaires relating to their stress perceptions, actual academic loads and their coping strategies. The main objective was to explore the effect of stress perceptions on coping behavior while accounting for objective loads and demographic parameters. Multilevel analyses revealed several indications: first, students’ coping behavior could be predicted from their reported stress perceptions and their appraisals of their academic-related stress levels; second, students employed mainly task- and emotion-oriented coping strategies; and finally, students’ age was a significant factor in determining their coping behavior. Our findings suggest that, in stressful environments, each of the coping strategies functions independently, with the type of strategy adopted depending largely on the specific profile of each student’s stress perceptions and demographic characteristics.

This study examined the relationships between stress and coping strategies among college students. Participants completed questionnaires relating to their stress perceptions, actual academic loads and their coping strategies. The main objective was to explore the effect of stress perceptions on coping behavior, while also accounting for objective loads and demographic parameters.

Sources of Academic Stress and its Likely Impact on Students

College students perceive academic life as stressful and demanding (Wan, 1992; Hammer, Grigsby & Woods, 1998) and report experiencing emotional and cognitive reactions to this stress, especially due to external pressures and self-imposed expectations (Misra & McKean, 2000). They report on numerous stressors during term-time, including academic demands and social adjustment.

Stress-inducing academic demands include grade competition; lack of time and issues relating to time or task management (Macan, Shahani, Dipboye & Phillips, 1990; Trueman., & Hartley, 1996); the need to adapt to new learning environments (van-Rooijens, 1986) in terms of the increased complexity of the material to be learned and the greater time and effort required to do so; and the need to constantly self-regulate and to develop better thinking skills, including learning to use specific learning techniques (Fram & Bonvillian, 2001). Emotional stress, such as anxiety, students’ appraisal of the stressfulness of the role’s demands and of their ability to cope with those demands (Wan, 1992), are also connected to academic stress.

Another category that evokes stress is social adjustment, particularly adjusting to university life (Saracoglu, Minden, & Wilchesky, 1989; Abouserie, 1994) and separating from family and friends. Finally, other constraints include financial pressure (Miech & Shanahan, 2000) and other technical difficulties.

Thus, academic stressors cover the whole area of learning and achieving in and adjusting to a new environment in which a great deal of content must be assimilated in a seemingly inadequate period of time. Since students endeavor to adapt themselves to academic life, positive adaptation and well-being factors are associated with fewer experienced stress symptoms (Van-Rooijen, 1986; Tobin & Carson, 1994).

Coping Strategies

Coping strategies are assumed to have two primary functions: managing the problem causing stress and governing emotions relating to those stressors (Folkman & Lazarus, 1980, 1986; Lazarus & Folkman 1984). Interpreting their results in terms of this assumption, most studies confirm two major related findings. The first is that a situation is evaluated as stressful, in part, whenever the individual perceives a lower ability to cope with it. The second finding is that stressors perceived as controllable elicit more proactive coping mechanisms (Karasek & Theorell, 1990), while those perceived as uncontrollable elicit more avoidance strategies (Anshel & Kaissidis, 1997; Compas, Malcarne & Fondacaro., 1988; Lazarus, 1981; Lazarus & Folkman, 1984; Roecker, Dubow & Donaldson, 1996).

Differences in the conceptualization of coping have led to a number of ways of classifying coping strategies. Lazarus and Folkman (1984) offered a widely used definition of coping, namely: constantly changing cognitive and behavioral efforts to manage specific external or internal demands. Subsequently, Higgins and Endler (1995) grouped coping strategies into three main classes: task-oriented, emotion-oriented, and avoidance-oriented.

The task-oriented strategy is problem-focused. It involves taking direct action to alter the situation itself to reduce the amount of stress it evokes. In the emotion-oriented strategy, efforts are directed at altering emotional responses to stressors. It also includes attempts to reframe the problem in such a way that it no longer evokes a negative emotional response and elicits less stress (Mattlin, 1990). Finally, avoidance-oriented coping includes strategies such as avoiding the situation, denying its existence, or losing hope (Lazarus & Folkman, 1984). It also includes the use of indirect efforts to adjust to stressors by distancing oneself, evading the problem, or engaging in unrelated activities for the purpose of reducing feelings of stress (Roth & Cohen, 1986).

The first two coping strategies involve pro-active efforts to alter the stressfulness of the situation, with the use of emotion- oriented strategies being favored by people whose personality disposition enables them to easily enter into and sustain a state of emotional arousal in response to, or in anticipation of, emotionally- laden events (Melamed, 1994). By contrast, avoidance strategies are characterized by the absence of attempts to alter the situation. The two proactive strategies, namely the task-oriented and emotion- oriented approaches, are associated with better adjustment, as reflected in higher self-rated coping effectiveness and less depression (Causey & Dubow, 1993; Compas et al., 1988; Moos, 1990; Reid, Dubow & Carey, 1995; Strutton & Lumpkin, 1993). Although avoidance-oriented coping may initially be an appropriate reaction to stress. Billings and Moos (1981) have shown that it is associated with poorer adjustment, and Endler and Parker (1999) have suggested that, in the long run, task-oriented coping is the most efficacious strategy.

Effects of Academic Stress and Demographics on Coping

Although a large body of literature has gauged the effects of academic stressors on coping strategies, little research has examined the importance of developing an integrative model, incorporating the effects of the perceived and actual stressor parameters on coping strategies.

With respect to the effect of academic stress on coping, the higher education literature shows that students’ coping methods are diverse, reflecting personal influences on their coping styles. Students generally report using proactive behavioral methods, such as managing their time, solving specific problems and seeking information and help (Misra & McKean, 2000; Britton, 1991; Lopez, Mauricio, Gormley, Simko & Berger, 2001; Collins, Mowbray & Bybee, 1999). Mattlin et al, (1990) found that students also use cognitive emotion-related behavior, such as positive reconceptualization of the stress-inducing events, to cope with stress.

Permeating these results we find demographic differences in coping styles. Researchers have found that ethnic, cultural (Kim, Won, Liu, Liu, &, Kitanishi, 1997) and even socioeconomic (Cairns, 1989) characteristics influenced coping behaviors. As for gender, Haarr and Morash (1999) found that significant differences come into play with respect to avoidancebased strategies, with women reporting a significantly higher level of use of avoidance than men. Other researchers found that males favor the use of task oriented methods and physical coping resources, and are more likely to endeavor to solve problems, while females are inclined to make more use of emotional and social coping resources (Rawson, Palmer & Henderson, 1999). Undergraduate male students who use task-oriented coping techniques report experiencing less distress (Higgins & Endler, 1995), while the use of emotion-oriented coping strategies was a significantly positive predictor of distress in both men and women.

Age has also been found as a factor that mediates stress levels. Studies that focused on perceived stress found that it decreases with age (Cohen & Williamson, 1988; Hamarat, Thompson, Zabrucky, Steele, & Matheny, 2001).

To summarize, studies of stress and coping offer only a partial demonstration of the coping strategies employed. In particular, the literature has viewed coping behaviors in relation to either ‘actual’ stress or perceived stress, without endeavoring to determine from which aspect the coping behaviors derive. To investigate this issue, an integrated model is required.

Conceptual Model of Coping Strategies

The proposed multilevel structural model (Figure 1) simultaneously defines multidimensional constructs of objective variables (academic load), subjective variables (stress perceptions) and relevant demographics, and tests their direct and indirect effects on coping strategies.

Figure 1

Multidimension model of objective Academic Loads, Stress Perceptio\ns, Demographic Characteristics and Coping Strategies

In this formulation, perceived and objective stress parameters are proposed to explain coping strategies. Accordingly, we formulated the following hypotheses:

Hypothesis 1: Academic stress perceptions are predicted by objective academic load variables.

Hypothesis 2: Perceived academic stress, objective academic loads and demographic characteristics are correlated with the types of coping strategies adopted by students.

Hypothesis 3: Academic loads predict the use of task-oriented coping strategies, academic stress perceptions predict the use of emotion-oriented coping strategies and demographic characteristics predict the use of avoidance coping strategies.

Method

Participants

The target population was students studying in Israeli academic institutions. Questionnaires were distributed to students during the 2002 academic semesters. Except for two negligible refusals, all students completed the questionnaires. Of the respondents, 153 (51.4 %) were female, 119 (43.8 %) were male and 11 (4%) did not identify their gender, comparable to the Israeli student distribution 2000/1 (56.5% women and 43.5% male, Central Bureau of Statistics 2002, No. 53, Table 8.33(1)). In terms of their marital status, 179 (63.3%) respondents were single, 98 (34.6%) were married and 5 (1.8%) were divorced. Almost a quarter of respondents (68, being 24.1%) had children: 26 (9.2%) had one child, 22 (7.8%) had two children and 20 (7.1%) had three or more children. Most respondents (173, being 61.1% of the sample) were childless while 42 (14.8%) did not answer the question. Distribution by academic degree sought indicated that 156 (55.2%) of the students were studying for their first degree and 127 (44.8%) were studying for their second degree. The average age of the respondents was 30.13 years (SD = 6.78), with a range of 41 years (from 20 to 61 years).

Procedure

We distributed the questionnaire to stratified samples of 283 students studying in national universities and colleges in Israel, who participated in the study voluntarily. Class lecturers and assistants distributed all questionnaires during class time. Academic institutions were therefore selected by non-random convenience sampling. T-tests were performed for gender and age distribution in the institutions. Results show no significant differences in terms of gender (t = -0.55, F= 1.19, p > 0.05) or age (t= -3.26, F = 0.67, p > 0.05). Therefore we treated the students as one group.

Measures

This study comprised three parts: (1) the students’ subjective assessment of the stress they experience i.e., perceived stress; (2) an investigation of the task- emotion-and avoidance-related coping strategies they adopt; (3) an objective assessment of their actual academic loads.

1. Perceived Stress

In accordance with Lazarus’s (1990) definition, perceived stress was defined as a condition subjectively experienced by respondents who identify an imbalance between demands addressed to them and the resources available to them to encounter these demands. It was assessed in terms of the students’ subjective experiences of their academic stress. The question was: “Would you please share with us your feelings of stress regarding your academic loads: How much stress do you feel due to your academic studies?” Students answered on a four-point Likert scale from not stressed at all (1) through very much stressed (4).

2. Task- Emotion and Avoidance-Oriented Coping strategies

Coping strategies were measured using the Coping Inventory for Stressful Situations (Endler & Parker, 1999). This is a 53 – item measure of coping style composed of three factors, (a) Task- oriented coping – its subscales tap active and offensive coping styles, stressing proactive responses to the stressors (e.g., “I focus on the problem and see how I can solve it”). (b) Emotion- oriented coping – this scale represents coping styles directed at altering negative emotional responses to stressors, such as negative thinking (e.g., “My efforts will surely fail”), lowered self confidence (e.g., “I cannot handle this problem”) or poor self image (e.g., “I am useless”). (c) Avoidance – this represents withdrawal behaviors and the redirection of personal resources towards different paths, such as sports, leisure time, etc. (e.g., “I buy something”). The scales for these three coping strategies range from 1 (seldom used) to 5 (always used). Higher scores represent a higher usage frequency for the specific coping strategy. Cronbach alpha coefficients obtained for the entire scale of coping strategies were: for task orientation, α = 0.89; emotional orientation, α = 0.87; avoidance, α = 0.83, indicating that the coping strategies questionnaire is a reliable measure of adult coping orientations for a college population.

3. Objective Stress Variables: Academic Loads

Academic loads were objectively assessed on an average weekly basis in terms of: (1) class hours; (2) study hours during semesters and (3) study hours during exam periods. Study hours included time spent in the library, in laboratories and at home, to meet academic demands.

Demographic variables

Data were collected with respect to each student’s age, gender and familial status. Since most of the students in our sample were not parents, familial status was not subsequently included in the model. This decision was supported by correlation calculations, which showed that the degree of correlation between familial status, academic stress perceptions and coping strategies was negligible.

Results

To investigate the first hypothesis, a regression analysis was performed on perceived academic stress, in order to evaluate the effects of objective stress parameters on stress perceptions. An ENTER method was used in each equation, with academic stress perceptions entered as the dependent variable and variables relating to academic loads entered as independent variables. The results (Table 1) showed that objective parameters related to academic stress significantly affect academic stress perceptions (R2 = 0.075, F (3,283) = 5.97, p

To investigate the second hypothesis a Pearson correlation analysis was performed. As shown in Table 2, certain coping behaviors were significantly correlated with perceived academic stress, so supporting our hypothesis. Specifically, the correlation of perceived academic stress with task-oriented behaviors was significantly negatively (r = -0.16, p

Table 1

Regression analysis for academic stress perception with academic loads parameters

These findings urged investigation of the third hypothesis, for which purpose hierarchical regression analyses were performed. Separate analyses were conducted using each of the three coping strategies as dependent variables and the three other factors (namely, academic loads, stress perceptions and demographic characteristics) as independent variables. We were interested in investigating whether academic loads predict the use of task oriented coping strategies, academic stress perceptions predict the use of emotion oriented coping strategies and demographic characteristics predict the use of avoidance coping strategies. For this purpose, the independent variables were entered in three steps in an ENTER procedure, in the following order: (1) demographic characteristics (2) academic load variables and (3) academic stress perceptions.

Table 2

Correlations between coping strategies and stress perceptions (N = 283)

The results (Table 3) for all equations were significant, indicating that each of the coping strategies was significantly predicted by the independent variables. It is notable that for both task and emotion oriented strategies, academic stress perceptions significantly contributed towards predicting coping behavior, notwithstanding that this variable was entered last. As hypothesized, academic stress perceptions affected these two coping behaviors in opposing ways. Thus, while academic stress perceptions significantly and positively predicted the use of emotion-oriented strategies (B = 0.25, p

A deeper examination of the regression equations for the emotion- oriented strategy reveals that the transition from step two (inclusion of academic loads, B = -7.47) to step three (inclusion of academic stress perceptions, B = 0.25) is relatively sharp and positive, indicating that stress perceptions make an important positive contribution to the prediction of emotionoriented strategies. This contribution is statistically significant (second step: R = 0.32, R^sup 2^ = 0.10; third step: R = 0.36, R^sup 2^ = 0.13). Furthermore, these results suggest that the greater the level of academic stress experienced, the more students tend to manage it through emotion-oriented coping strategies. A similar examination of the regression equations with respect to the task oriented coping strategy shows the opposite result, in that the sharp transition from step 2 to step 3 is negative. Thus, while objective load variables shift students towards the adoption of task-related coping behaviors (B = 7.28; p = 0.05), the subsequent inclusion of aca\demic stress into the equations reverses the results (B = -0.21 ; p = 0.00). These findings suggest that, initially, students tend to use task-oriented strategies to manage their objective academic loads. Having done so, they subsequently refrain from using these strategies and focus on managing any remaining academic stress perceptions.

Table 3

Hierarchical Regression Analyses for Coping Strategies with the Independent Variables: (1) Demographic Characteristics, (2) Academic Loads and (3) Stress Perceptions

Other findings of interest discerned from the analyses relate to the demographic characteristics. Age has been found to be a substantial variable appearing as a significant predictor for most of the coping behaviors. The scores show that older students employ task-oriented techniques in preference to any other coping strategy, while younger students also employed emotion-oriented and avoidance strategies. We found gender to be a significant variable only with respect to the avoidance coping strategy, with males adopting this coping strategy more than females.

Discussion

We surveyed a varied sample of university and college students to examine three hypotheses concerning the coping strategies employed by students in response to different types of stress. We were particularly interested in elucidating the role of academic perceived stress versus academic objective loads in shaping the coping strategy used.

As we hypothesized, both academic stress perceptions and academic loads had significant and unique effects on students’ coping strategies. The experience of academic stress was mainly associated with the use of emotion-oriented behaviors, while being significantly negatively related to adoption of task-oriented strategies. This indicates that the nature of the stress perception can also be significant in restraining the use of certain coping behaviors. Moreover, objective and subjective stress experiences fulfilled opposite roles in the prediction of coping behavior. In particular, the subjective perception of academic stress acted as a restraining factor in students’ employment of task-orientated coping behaviors, while objective academic loads supported the use of this coping strategy.

The statistical coefficient scores suggest that most academic stress perceptions derive from actual academic loads. We further find that both of these factors are addressed by working students through proactive means. Academic load is addressed principally through a task orientation and academic stress primarily through an emotion-oriented strategy. The literature shows that proactive strategies are preferred in situations that are, or are perceived to be, controllable (Karasek & Theorell, 1990) and that students generally utilize proactive behavioral methods to manage academic stress (Misra & McKean, 2000; Britton & Tesser, 1991; Lopez et al, 2001; Collins et al, 1999). Interpreted in this light, our data suggest that students perceive the academic component of their stress to be at least partly controllable. Therefore, they initially address academic stress through task-oriented behaviors. As actual academic loads reduce, but the perception of stress remains, task- oriented coping techniques lose their relevance, and emotion- oriented behaviors predominate. This interpretation is also partially supported by the work of Mandler (1993), who suggests that individuals’ reaction to stress takes two forms. First, individuals ruminate about the stressful situation, their actions proceeding automatically from the way they interpret it, in accordance with their customary, learned behavioral patterns. Then, if this does not yield a solution to the problem (i.e., the perception of stress remains), emotional and affective reactions arise. .

In agreement with Mattlin et al (1990), we find the utilization of emotion-oriented strategies to be affected by academic loads. This may be explained by the adaptive functions of emotions in managing stressful situations, [such as are generated by heavy academic loads,] by preparing the individual for more effective thought and action, for example (Mandler, 1993).

The data also show that women and men employ a wide array of similar strategies to deal with stress. Gender effects did not appear even in examinations of the interactions between stress perceptions, coping strategies and gender. Significant gender differences come into play only with respect to the avoidance coping strategy, with men reporting a significantly higher usage of avoidance as a coping tool. This finding is inconsistent with much of the stress and coping literature, in which distinct gender-based coping behaviors are well established for all coping strategies, and with women reporting a significantly higher level of use of avoidance than men (Haarr & Morash, 1999).

Consistently with the literature (Cohen & Williamson, 1988; Hamarat et al, 2001), older working students’ coping appears task- oriented and they do not utilize indirect coping techniques. By contrast, younger students generally choose to manage stress by either emotion-oriented strategies or avoidance.

Overall, and in agreement with our hypotheses, we find that students’ academic stress perceptions can be predicted from their objective academic load variables. Furthermore, perceived academic stress, objective academic loads and demographic characteristics are correlated with the types of coping strategy adopted by students, with academic loads predicting the use of task oriented coping strategies, academic stress predicting the use of emotion-oriented coping strategies, and age and gender (demographic characteristics) predicting avoidance reactions. Thus, the data support our proposed model. The main implication of the results is that students who face stressful situations choose to deal with them through a “step-by- step” coping strategy. As such, they initially adopt a task-focused approach to manage their actual loads to reduce stress associated with phenomena that they consider controllable. They then utilize indirect emotion-oriented techniques to address residual perceived stress.

1 Title: Students in Universities, by Degree, Field of Study and Institutions.

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DAFNA KARIV, PH.D.

School of Business Administration The College of Management

TALI HEIMAN, PH.D.

Department of Education and Psychology The Open University of Israel

Copyright Project Innovation, Inc. Mar 2005

The Evaluation of Organization Development Interventions: An Empirical Study

Abstract

A survey of Fortune 500 HR Executives was used to test seven hypotheses concerning the evaluation of organization development, interventions. Among the findings are uncertainty in the intervention creates the client’s expectation for multiple-level evaluations, and idiosyncratic investment in an intervention effects the desired level of evaluation rigor.

Organization Development (OD) is one of the rare exceptions for which businesses consistently pay out large sums of money for services but fail to determine if the services obtained are satisfactory (Jones, Spier, Goodstein & Sashkin, 1980). One survey, albeit dated, of OD consultants indicated only 30% conduct evaluations more rigorous than a simple “gut instinct” determination that the intervention worked (Kegan, 1982). A recent critique of evaluation practices (Head, in press) supports the belief that very little has changed with regards to the frequency of rigorous evaluations.

Evaluation is a critical step in the OD process, appearing in almost all the process models (French, 1989). Cummings and Worley (1997, p. 30) make the case very succinctly:

Managers investing resources in OD efforts are increasingly being held accountable for results. They are being asked to justify the expenditures in terms of hard, bottom line outcomes more and more, and are using the results to make important resource allocation decisions about OD.

Evaluation provides the evidence on which to base decisions about maintaining, institutionalizing, and expanding successful programs, and modifying or abandoning unsuccessful ones (Weiss, 1972; Weissbord, 1973). Rossi and Freeman (1982) are more succinct regarding its purpose: Evaluation assures that the client “got what it paid for” (p. 16). There are two additional reasons to evaluate one’s work (Head, 2004). It is important to determine whether unforeseen problems arose as a consequence of the intervention. Without appropriate evaluation there is no way that an organization may be sure of a trouble-free intervention. Finally, until OD begins to gather solid evidence for its “results,” it will not be truly taken seriously as either a science or a body of knowledge. Science grows upon established facts, and no amount of rhetoric, or “gut instinct speculation,” however well-intentioned, will change this.

While failure to evaluate is a significant issue, there also appears to be a contradicting problem. Rutman and Mowbray (1983) believe that some clients might demand, and subsequently pay for, much more evaluation than is logically required. The problems that arise from this approach are fairly significant. First, evaluation is costly, and any unnecessary evaluation represents unwarranted expense. Second, processing evaluation data takes time, which can delay communicating critical information to the client. Finally, too much evaluation could represent an over-dependence on data in subsequent decision-making. In light of these consequences of overevaluating, it behooves both client and consultant to have some decision rules to guide in establishing the needed level of rigor.

Head and Sorensen (1990) proposed eight propositions to act as such a guide for establishing how much and what type of evaluation should be conducted. These propositions emerged through a review of various literatures tangentially related to OD, such as transaction costs economics and community and social agency program evaluation. Seven of these untested propositions serve as the hypotheses for this current study.

Head and Sorensen (1990) used Kirkpatrick’s (1960) four-level hierarchical training evaluation model as a base for their work. Kirkpatrick posits four evaluation levels arranged by increasing rigor (and cost). The selection of one level does not preclude, or require, the selection of any other; evaluation may take many forms, depending upon which levels the client desires to monitor, and how much it wishes to spend. Evaluation’s lowest level is reaction, measuring how the employees involved in the intervention perceived the experience (such as a satisfaction survey). The second level is learning, where the consultant determines whether or not the employees gained the new system’s requisite knowledge and understanding, often times through a test, or “dry run”. Behavior is the third level, and involves establishing whether or not the intervention altered the employees’ actions and behaviors in the desired direction. Typically, this level requires direct observation and pre/post measurements. The most rigorous evaluation level is that of results. At this level the consultant establishes the intervention’s impact on the client’s bottom-line performance (cost/ benefit analysis, long-term gains, and the like).

The original work by Head and Sorensen (1990) offers a complete discussion of how the current hypotheses were developed. Presented here, as research hypotheses, are seven of the eight propositions along with a brief summary of the logic for each.

The first proposition addresses uncertainty in the consulting situation. The greater the uncertainty in terms of problem identification and subsequent solution parameters, the more flexibility for action is required by the consultant, and the contract will have to be vague with regard to processes. However, this freedom to act creates a greater need to establish accountability. Therefore:

Hypothesis 1: The greater the uncertainty around the intervention, the more rigor will be demanded by the client in evaluations.

If a consultant has been frequently used by a client, a high degree of trust and confidence is likely to have been established between both parties, and the client will require less evaluation:

Hypothesis 2: The more frequently a client organization has used a specific consultant, the less rigor will be required in evaluation.

Often times clients are, by using a consultant, obligated to incur expenses for items that cannot be used for any other purpose, or by any other consultant. The more the client is expected to make such idiosyncratic investments, the more rigorous an evaluation will be required:

Hypothesis 3: The more the organization is required to invest in the intervention, the greater the likelihood that rigorous and multiple level evaluations are to be required.

With a “single-shot” intervention, one not intended to be repeated, the client’s interest focuses on whether or not the project worked. However, if the intervention under review is a pilot project to be replicated throughout the organization, then both parties will be interested in the intervention’s implementation process as well as its results. Therefore:

Hypothesis 4: If the intervention is intended to be used frequently by the organization, the initial evaluation will stress reaction and learning. But if the intervention is not intended for replication, the evaluation will stress behavior and results levels.

Typically, variables at the behavior and results level require a substantial period of time to lapse after the intervention to show any significant change. At the same time, for various reasons, particularly political, the client often requires quick evaluations. Therefore:

Hypothesis 5: The faster evaluation information is needed, regardless of the reason, the less rigorous the evaluation will be.

On the other hand, if an intervention has been performed several times in the client’s organization, it can be assumed to be reliable and that all the process “bugs” have been resolved. At the same time it should be relatively easy to determine long-term effects, as it is no longer an isolated incident:

Hypothesis 6: If an intervention has been performed frequently in an organization, then a results level evaluation will be the only evaluation requirement.

Finally, most behavior and results level variables require classical pre-test and post-test comparisons. Therefore, they require extensive planning and must be identified prior to any intervention’s implementation.

Hypothesis 7: If the intervention is initiated prior to the fmalization of evaluation plans, it is less likely that behavior and results levels of evaluation will be required.

Methodology

Survey Development

Two one-paragraph cases were written for each hypothesis. The cases represent opposite ends of the issue under study. For example, hypothesis 3 examines the impact of client investment. The first case involves a client investment of $300 and the second involves the expenditure of $7500 for materials. Each case was then randomly assigned to one of two survey forms.

The survey cover page included a description of the four levels of evaluation (reaction, learning, behavior, results), some examples of how each might be measured, and clearly established that the levels are not mutually exclusive. Next, the seven cases were presented. Following each case were questions in which respondents were asked to identify the types of evaluation they would require from the OD consultant. Respondents were also asked what percentage of the consultant’s fee they would like to see dedicated to the evaluation process. Because only 30 percent of the respondents completed the fee percentage questions, this data was excluded from the ana\lysis.

The two survey forms were reviewed independently by five independent OD consultants. These individuals examined the survey for clarity, realism, and certainty that the cases were significant opposites. Based upon the reviewers’ feedback, the cases were revised. The final versions of the cases are located in the appendix.

Subjects

Two hundred and fifty of each survey form, accompanied by a postage-paid return envelope, were mailed to Fortune 500 Human Resource Executives. The highest level HR individual from each company’s corporate office, or a large division, that could be identified was selected to receive the survey. Generally the surveys were mailed to the corporate vice president. The specific survey form each subject received was randomly assigned.

Analysis

The survey resulted in two related sets of data. The first set involved the specific levels of evaluation selected for each case by the respondents. The second involved the number of evaluation levels the respondents selected for each case. The hypotheses were evaluated using Z tests on the transformed percentages.

Results

Of the 500 mailed, 94 usable surveys were received, for a return rate of 18.9 percent. One form resulted in 52 returns (20.8 %) and the other form had 42 responses (16.8 %). It is important to remember in interpreting the results that the frequencies reflect what kinds of evaluations the respondents believed they would require from a consultant, and not what they actually did require in the past.

Hypothesis one stated that the more uncertainty surrounds a consulting situation, the greater the rigor that will be required from the evaluation. Therefore, in an ambiguous situation, one should expect multiple-level evaluations, while clear situations would require only one or two levels. There should also be a greater emphasis on results and behavior in ambiguous situations than in clear situations.

The survey results for hypothesis one (Table 1) show general support for the hypothesis. Significantly, more executives required evaluation at all four levels (Z = 3.463, p

Contrary to what was expected, no significant difference was found at the behavior level. However, as predicted, evaluation at the results level was required significantly (Z = 2.146, p

Hypothesis two revolved around the belief that a client will, over time, develop a high level of trust in a consultant and as a result will require less in terms of evaluations from him or her. This trust would manifest itself in fewer levels being expected and an emphasis on the more basic levels of reaction and learning.

Results of the analysis for hypothesis two are found in Table 2. The table shows mixed results. As predicted, having a great deal of experience with a consultant resulted in fewer, or no, levels of evaluation being required (1 : Z = 2.406, p

Evaluation at the reaction level was required significantly more often (Z = 3.272, p

Hypothesis three predicts that the greater an idiosyncratic investment is required from a client for a given consultant’s intervention, the more rigorous (behavior and results levels), and more levels of, evaluation will be required. The data, located in Table 3, strongly supports hypothesis three.

Table 1. Results for Hypothesis One Uncertainty in Consulting Situation Type and Number of Evaluation Levels Selected

When clients had to make a large idiosyncratic ($7500 in material) investment they did tend to require multiple level evaluations (4: Z = 1.776, p

While there were no differences between the two conditions at the results and learning levels, the other two levels did support the hypothesis. Reaction (Z = 4.48, p

Table 2. Results for Hypothesis Two Client Experience with the Consultant Type and Number of Evaluation Levels Selected

Table 3. Results for Hypothesis Three Client Investment in the Intervention Type and Number of Evaluation Levels Selected

Hypothesis four has two dimensions. With an intervention, which is not intended to be replicated, or a “single shot,” the client will be interested only in the outcomes (results and behavior levels). Clients will be more interested in the intervention’s process (reaction and learning levels) for a pilot intervention that is intended to be used frequently by the organization.

The results for hypothesis four, located in Table 4, strongly contradict the hypothesis. It was clear that evaluation was much more important for the pilot project than for the single shot intervention. In fact, significantly more subjects reported not requiring any evaluation (Z = 2.666,p

Table 4. Results for Hypothesis Four Intended Frequency of the Intervention Type and Number of Evaluation Levels Selected

Hypothesis five addresses the impracticality of requiring rigorous evaluations soon after an intervention’s implementation, as changes at the behavior and results levels require additional time to emerge. It is believed that evaluations needed immediately will stress reaction and learning, and evaluations with no such rush will tend to involve more levels and specifically include behavior and results.

Table 5. Results for Hypothesis Five Speed with Which Evaluation is Required Type and Number of Evaluation Levels Selected

Table 5 presents the results for hypothesis five. There appears to be mixed support. The type of evaluation selected is completely in line with expectations. Results (Z = 4.962, p

However only one significant trend emerged with regards to the number of evaluation levels clients expected, and this was contrary to the prediction. When the evaluation was not needed immediately, significantly fewer respondents (Z = 2.664, p

The results level, according to hypothesis six, will be the only concern for clients who have frequently used a particular intervention. This assumption is based upon the belief that any process bugs will have been resolved and the client is comfortable with the reaction, learning, and behavior levels (otherwise the intervention would not have been repeatedly implemented).

able 6 contains the results for hypothesis six. There was no significant difference for the results level between the conditions. In fact, one significant difference that occurred was completely opposite of the hypothesized prediction. When the client was experienced with the intervention, significantly more subjects required evaluation at the reaction level.

Hypothesis seven reflects the need for behavior and result-level evaluations to have pre-intervention baseline data available or comparison purposes. If an intervention is initiated prior to the finalization of evaluation plans, any evaluation at these two most rigorous levels may be suspect, and as a result not desired.

Table 6. Results for Hypothesis Six Frequency of Intervention Implementation Type and Number of Evaluation Levels Selected

Hypothesis seven received partial support as can be seen from the results located in Table 7. There was no significant difference, based upon whether the intervention began before the evaluation plan was established, at the behavior level. However, s\ignificantly more (Z = 4.046, p

Table 7. Results for Hypothesis Seven Intervention Implemented Prior to Evaluation Plan Type and Number of Evaluation Levels Selected

Discussion

It is clear that organization development consultants must embrace evaluation as a necessary step in the action research process. The field cannot progress in knowledge and reputation without practitioners being able to “back up” claims of success with clear and hard data. In order to make these claims, we, as a field, must first gain insight into what criteria should drive our selection of evaluation data. The key to an effective evaluation is to provide the client with relevant information. Relevance, here, refers to data on the specific dimensions required by the client for future decision-making. It is critical to avoid both information underload and overload. Too little information and the client makes decisions “in the dark.” Too much information and not only is money wasted, but the client might be distracted by irrelevant information.

Seven theoretical propositions, based upon Kirkpatrick’s four levels of training evaluation, were tested using surveys received from 94 Fortune 500 HR executives. The first observation is that clearly executives want to see OD interventions evaluated in some manner. It also appears that straightforward logic, such as used in developing these propositions, is a (but not the only) driving force behind deciding on the exact form the evaluation takes. The OD consultant can also be comforted in knowing that in general, clients do recognize that often times specific intervention conditions will place constraints on the evaluation process.

Regarding the specific hypotheses the results indicate:

1. Uncertainty in the consulting situation leads to the desire for multiple-level evaluation with an emphasis at the results (bottom-line) level.

2. A frequently used consultant will either not be required to evaluate his or her work by the client, or at most will have to evaluate at only one or two levels.

3. The larger an idiosyncratic investment a client incurs in an intervention, the more likely it will require multiple-level evaluations, particularly emphasizing the “hard results” areas of reaction and behavior. Consultants who require employees to take expensive “off-the-shelf diagnostic surveys and training materials will find this result particularly important. This observation is not a criticism of such tools, rather an acknowledgement that they do represent idiosyncratic investments.

4. One time, or “single shot,” interventions are more likely not to be evaluated or evaluated at only one level, but “pilot” interventions that are anticipated to be replicated throughout the organization will typically require evaluations at all levels as the client seeks assurance that the intervention’s process was appropriate. In other words, the “means to the end” are just as important as the end results themselves.

5. When evaluation data are not required soon after program implementation, clients will expect data on both the results and behavior levels. When the evaluation is needed in a hurried fashion, the clients will tend to expect less rigorous data focusing upon reaction and learning levels. This finding supports the notion that clients recognize time is needed “to do it right” and that consultants will not be expected to do the impossible. However it does clearly establish that when given time, the consultant is expected to perform a rigorous evaluation.

6. A frequently used intervention in the client’s organization appears to require only reaction-level evaluations. The clients are confident that the intervention produces positive results, and therefore do not wish to pay for obtaining knowledge they already possess. At the same time, obtaining the inexpensive and easy reaction data can simply be their way of keeping an eye on the process to detect any anomalies or issues.

7. When an evaluation plan has been established prior to an intervention’s implementation, the client will expect rigorous, multiple-level, assessments with specific interest on the results level. This finding acknowledges that, for various reasons, OD projects do not always follow the action research model. However, it also establishes that when the consultant is able to conform to action research, it includes the essential evaluation step.

An interesting final issue that was raised by several of the respondents must be noted. This study, and in fact the majority of the evaluation literature, focuses upon evaluations conducted by the consultant. The fact that the client may conduct its own evaluation, or the only evaluation, was not considered, but appears to be a viable, and understandable, option.

This oversight raises many questions for future organization development model building. While evaluation is an essential element of the action research model, there is no requirement as to who must perform it. It has always been assumed that the consultant is in the best position to judge success, much like a physician treating the ill. Is this a valid analogy for organization development? When one purchases a product or service, do we typically leave it to the salesperson/provider to determine our satisfaction with the purchase?

There are several questions that OD’s future models need to examine. When and why would a client choose to conduct a self- evaluation? When and why would a client choose to rely on a consultant to conduct an evaluation? When and why would a client have both parties evaluate? Would this decision involve duplication or separate dimensions?

This paper has provided some direction and answered some questions, but it is possible that more issues have been raised than resolved, highlighting the need for organization development, as a field, to evaluate its models and activities, just as intervention evaluation is required. After all, a field that focuses entirely upon implementing change in others must itself be willing to embrace change as well.

Evaluation is a critical step in the OD process.

Some clients might demand, and subsequently pay for, much more evaluation than is logically required.

Head and Sorensen (1990) used Kirkpatrick’s (1960) four-level hierarchical training evaluation model as a base for their work.

The more uncertainty surrounds a consulting situation, the greater the rigor that will be required from the evaluation.

While evaluation is an essential element of the action research model, there is no requirement as to who must perform it.

References

Cummings, T. & Worley, C. (1997). Organization development and change, 6th ed., Cincinnati: Southwestern Publishing.

French, W. (1989) A checklist for organizing and implementing an OD effort. In W. French, C. Bell & R. Zawacki (Eds.), Organization Development: Theory, Practice & Research, 3rd ed. (522-532). Homewood, IL: Irwin Publishing.

Head, T. (2004). The current state of evaluation in organization development practice: A harsh critique. Pro Change International Journal, 1, in press.

Head, T. & Sorensen, P. (1990). Critical contingencies for the evaluation of OD interventions. Organization Development Journal, 8, 58-63.

Jones, J., Spier, M., Goodstein, L. & Sashkin, M. (1981). OD in the 80s: Preliminary projections and comparisons. Group and Organization Studies, 5, 5-17.

Kegan, D. (1982). Organization development as OD network members see it. Group and Organization Studies, 7, 5-11.

Kirkpatrick, D. (1969). Techniques for evaluating training programs, Training and Development Journal, 13, 3-9.

Rossi, P. & Freeman, H. (1982). Evaluation: A systematic approach. Beverly Hills, CA: Sage Publications.

Rutman, L. & Mobray, G. (1983). Understanding program evaluation. Beverly Hills, CA: Sage Publications.

Weisbord, M. (1973). The OD contract. OD Practitioner, 5, 1-4.

Weiss, C. (1972). Evaluating action programs. Englewood Cliffs, NJ: Prentice Hall.

Woodman, R. (1989). Evaluation research on organizational change: Arguments for a “combined paradigm” approach. Research in Organizational Change and Development, 3, 161-180.

Thomas C. Head, PhD

Peter F. Sorensen, Jr., PhD

Thomas C. Head, PhD

Thomas C. Head, PhD, is Professor of Management in the Heller College of Business, Roosevelt University. Tom has worked on projects for a wide range of clients from Fortune 15 to small start- ups. He has authored 13 books and over 50 scholarly articles. Tom is currently on the board of Pro-Change International and is the Region 4 President Elect for the ACBSP. His PhD is in Business Administration from Texas A&M University.

Contact Information

Walter E. Heller College of Business Administration

Roosevelt University

1400 N. Roosevelt Blvd.

Schaumburg, IL 60173

(847) 619-4866

[email protected]

Peter F. Sorensen, Jr., PhD

Peter F. Sorensen, Jr., PhD, is Director of the PhD program in OD and the MOB program at Benedictine University. In 2002 he was Chair of the ODC Division of Academy of Management. Peter was an invited, distinguished scholar to the first Academy of Management Conference on Global Change, and has received the “Outstanding OD Consultant of the Year Award” from the OD Institute.

Contact Information

Organizational Development Doctoral Program

Benedictine University

5700 College Rd.

Lisle, IL 60532

(630) 829-6222

[email protected]

Appendix

Cases for Hypothesis 1

“You have recently hired a consultant to conduct employee workshops in o\rder to solve a problem. It was fairly easy to discover and completely agree on the workshop’s content.”

“You have recently hired a consultant to conduct employee workshops in order to solve a problem. You are aware there is a problem; however, you are not sure exactly what it is, or what form the workshops will take. You have given the consultant great discretion in terms of the workshop’s content and duration.”

Cases for Hypothesis 2

“You have just hired a consultant for a department turnaround project. You have frequently used this consultant and she has proven very successful in the past.”

“You have just hired a consultant for a department turnaround project. While you have heard good things about this consultant, she has not worked for you before.”

Cases for Hypothesis 3

“The consultant that you have been working with on a fairly large change project has required you to purchase $7500 in materials that you’re sure can’t be used for anything other than the consultant’s project.”

“The consultant that you have been working with on a fairly large change project has required you to purchase $300 in materials that you’re sure can’t be used for anything other than the consultant’s project.”

Cases for Hypothesis 4

“You are just finishing a one-shot project with a consultant. Regardless of how well the project works there is no intention to repeat it anywhere else.”

The project that you and a consultant have been working on is a pilot study. If all goes well it will serve as a model for similar projects in other plants.”

Cases for Hypothesis 5

“You have been told by the consultant that while a ‘current state of affairs’ is possible now, it would require at least four months for any accurate long-term trends to emerge from a just-completed project. The president tells you that while he does want a report, time is not of the essence.”

You have been told by the president that a report on a project’s outcome will be needed immediately after the project’s completion. The consultant has told you that while a ‘current state of affairs’ is possible, long-term predictions are not practical in so short a time.”

Cases for Hypothesis 6

“Your consultant is just finishing another department’s communication workshop. Almost all of the departments have now received the workshop.”

“Your consultant is just finishing the second department’s communication workshop. The plan is that each department will eventually receive the workshop.”

Cases for Hypothesis 7

“Because of the immediacy of the situation, you had the organization development consultant begin making changes quickly. In fact changes were implemented before the consultant could obtain baseline data making before/after comparisons impossible.”

“Because of the immediacy of the situation, you had the organization development consultant begin making changes quickly. Fortunately the consultant was able to obtain baseline data so (if you wish) before/after comparisons are possible.”

Copyright O D Institute Spring 2005

Behind the Sensationalism: Images of a Decaying Corpse in Japanese Buddhist Art

One of the most provocative images in Japanese art is the kusozu, a graphic depiction of a corpse in the process of decay and decomposition. The kusozu, “painting of the nine stages of a decaying corpse” (hereafter, painting of the nine stages), was executed in Japan from approximately the thirteenth through the nineteenth centuries in various formats, including handscrolls, hanging scrolls, and printed books. The subject itself is derived from a traditional Buddhist doctrine that urges contemplation on the nine stages of a decaying corpse (kusokan, hereafter, contemplation on the nine stages). The teaching dates to the early fifth century and promotes a systematic meditation on the impurity of a decaying corpse as an aid to ardent devotees who wish to liberate themselves from sensual desires and affections.1

This paper explores unrecognized features of the paintings of the nine stages as they appear through almost half a millennium of Japanese art. We will see that these narrative paintings functioned as distinct visual agents for audiences in different eras. The functionality of the image shifted from a meditative focus for pietistic catharsis, to a didactic incentive for the pursuit of paradise, to an intercessory offering for the dead at merit transferal rites, to a popularized platform for politically manipulated precepts on feminine morality. After giving the textual and theological background for the nine stages of a decaying corpse, I will examine four images of the nine stages from different centuries, which I term the Nakainura, Raigoji, Dainenbutsuji, and Akagi versions. Finally, some remarks are offered on the enduring vitality of this sensational subject.

Religious and Literary Background

A proper understanding of these images relies on a conversance with the doctrinal sources treating the decaying corpse as a subject for devotional practice. The contemplation on the nine stages, while found in many Buddhist sutras, first appeared in the Sutra on the Samadhi Contemplation of the Ocean like Buddha (Japanese: Kanbutsu zanmai kaikyo; Chinese: Guanfo sanmei hai jing, translated by Buddhabhadra [359-429], ca. 400) or the Discourse on the Greal Wisdom (Japanese: Dai chidoron; Sanskrit: Mahaprajnaparamitita- sastra, translated by Kumarajiva [344-413], 402-5).2 The practice is a type of contemplation on impurity (Japanese: fujokan) that allows devotees to overcome hindrances to enlightenment and to conquer carnal desires, especially the sexual appetite.! In the Discourse on the Great Wisdom and other texts, such as the Chapters on the True Meaning of Mahayana Teachings (Japanese: Daijo gisho, Chinese: Dacheng yizhang), by the Chinese monk Hui Yuan (523-592), the love for another’s body is subdivided into multiple types, and instruction is given as to which phase of the decaying process is effective as a focus of meditation for conquering each lust.4

The Discourse on the Great Wisdom is especially significant in that this text contributed to the development of the contemplation on the nine stages and its pictorialization in Japan. This text provided the canonical sequence of corporeal decay used in paintings of the genre (see App. 1): (1) distension (choso); (2) rupture (kaiso); (3) exudation of blood (ketsuzuso); (4) putrefaction (noranso); (5) discoloration and desiccation (seioso); (6) consumption by animals and birds (lanso); (7) dismemberment (sanso); (8) bones (kosso); and (9) parched to dust (shoso). The order stated in the Discourse on the Great Wisdom probably entered Japanese paintings of the subject by means of the Discourse on Mahayana Meditation and Contemplation (Japanese: Malta shikan, Chinese: Molie zhiguan), recorded and edited by a disciple of the Chinese Tiantai (Japanese: Tendai) master Zhiyi (538-597) based on his lecture in 594. The Discourse on Mahayana Meditation and Contemplation, which preserved the order of decay given in the Discourse on the Great Wisdom, had a substantial impact on Ptire Land Buddhist belief, including the content of the Essentials of Salvation (Japanese: Ojoyosku), a seminal work of Pure Land belief written by the Japanese monk Genshin (942-1017) of the Tendai school in 985.5 The description of the stages of a decaying corpse in the Essentials of Salvation includes direct quotations from the Discourse on Mahayana Meditation and Contemplation, although the order of the stages differs (see App. 1).

The nine stages of decay appear also in medieval literature, and a review of these passages can further illuminate the development of the subject. The theme is found in a verse form termed “poem of the contemplations on the nine stages of a decaying corpse” (Japanese: kusokanshi). Such poems have survived in China as well as in Japan,6 where two versions existed. The Japanese poems were written in Chinese characters and are attributed to the luminaries Kukai (774- 835) and Su Tongpo (1063-1101).7 The two poems (which can be termed the Kukai and Su Tongpo versions) derive their authority from the stature of their supposed authors: Kukai, the famed Japanese monk who brought esoteric Buddhism from China to Japan and founded the Shingon school, and Su Tongpo, who was a Northern Song scholar- bureaucrat as well as a renowned poet and calligrapher. Both poems detail the nine stages of a decaying corpse, but in different formats and orders, and with some variation in the designations assigned to each stage (see App. 1). The Kukai version has a short preface followed by twelve five-character verses for each stage, while the Su Tongpo version has a preface followed by eight seven- character verses for each stage. The Su Tongpo poem is included in woodblock-printed books dated between 1380 and 13849 and in depictions of the nine stages that were widely circulated during the Edo period (1603-1867), with examples found in printed books, hanging scrolls, and handscrolls. (Because of its importance to the depictions of the nine stages, the poem is translated in Appendix 2.) The oldest surviving illustrated handscroll inscribed with the Su Tongpo poem is dated to 1527 (at Dainenbutsuji in Osaka). As early as 1380, the poem began to be consistently accompanied by waka poetry, a verse form characterized by thirtyone syllables in five strophes. For each of the nine stages, the relevant verses of the Su Tongpo poem were followed by two waka, yielding a total of eighteen waka for the image. The author and date of these waka are unknown. The Kukai version, by contrast, never appeared in conjunction with the images of corporeal decay.

As it was frequently mentioned in Buddhist sutras, the practice of contemplating on a decaying corpse was adopted widely by monks regardless of their sectarian affiliations. Some medieval tales give accounts of contemplation on a decaying corpse and reveal how monks may have performed the practice with visual aids. For example, a tale in A Companion in Solitude (Kankyo no tomo, 1216), written by Keisen (d. 1296), describes an anonymous monk at Mount Hiei, headquarters of the Tendai school, who disappeared every night until morning.10 It was thought that he might be having relations with a woman, because he always looked sad on his return. One evening the monk was followed, and it was discovered that he was going down to Rendaino, a region renowned for its cemeteries, to meditate on a decomposing corpse.11 At this time, corpses were typically left exposed in cemeteries or in fields, since the practice of interment did not become widespread in Japan until after the fourteenth century.12 The medieval account provides anecdotal confirmation that Buddhist monks exercised the method of contemplation on the nine stages as taught in the Discourse on the Great Annotations of Abhidharma (Japanese: Abi daruma dai bibasharon, Sanskrit: Abhidharma-mahavibhasa-sastra, translated by Xuanzang, 656-59). The procedure is outlined in this sutra as follows:

Practitioner, first go to a mound to observe the stages of a decaying corpse, such as the stage of turning bluish black; for a deeper contemplation, step back and sit at a place and contemplate the image again. If the concentration is distracted and the image is unclear, and you wish to attain a better contemplation, again go to the mound to see it as before.13

Another literary example of a pious monk curbing his sensual desires through contemplation on an impure decaying body is found in A Collection of Religious Awakenings (Hosshinshu), written by Kamo no Chomei (d. 1216). It tells the story of the monk Genpin, who fell in love with the wife of a chief councillor at first sight and confessed this to the councillor. Since the chief councillor greatly respected Genpin, he arranged a rendezvous for the monk and his wife. Genpin appeared in formal clerical attire for their meeting. He never attempted to approach the woman but only gazed on her for about two hours and then left. The chief councillor’s reverence for Genpin deepened, seeing that the pious monk overcame his sensual desires by contemplating on the process of decay of the impure body of a beautiful woman. As a result of his contemplation, Genpin achieved an enlightenment in which he realized that people incapable of such self-control who indulge in transitory se\nsual desires lose their critical faculties, for attachment to the body is akin to relishing the droppings of maggots in a toilet.14 The narrative highlights how meditation on the decay of a corpse proved successful in eliminating a monk’s sensual desire for the female body through a realization of the transitory qualities of the human “shell.” The story also suggests that the trained monk had refined the skill of acquiring the mental image of a decaying corpse without the employment of a putrescent body. Accounts such as these show that contemplations on the corpse were a valued monastic practice in the pursuit of the pious life and that the decaying corpse served as a visual aid to the discipline, at least until the mental image of decay was attained and available for devotional practice.15 No Japanese medieval tales mention the use of pictorial images of the decaying corpse for meditation. Yet Chinese documents recount that such images accompanied the contemplation on the nine stages, and the practice likely was known among Japanese monks as well. For example, Zhiyi’s commentary on the Lotus Sutra, the Essential Meanings of the Lotus Sutra (Japanese: Myoho rengekyo gengi, Chinese: Miaofa lianhuajing xuanyi, 593), remarks that “[the high rank of] joyugi [Chinese: changyouxi] was given [after death] to those who have built a meditation hall for zen [meditation] practitioners and have painted the image of a corpse for contemplation.”16 Another example is that of the Chinese poet Baoji (active about Genso Tenpo [Chinese: Xuanzong Tianbao] 6 [747]) of the Tang dynasty (618-907), who composed a work entitled “Contemplation on the Mural of the Nine Stages of a Decaying Corpse.”17 The mural has not survived, but the title reveals that the practice of meditating on corporeal decay also existed in China. It is worth noting that Baoji composed a farewell poem for a Japanese envoy to Tang China, which means that the method of employing these paintings for devotional contemplation could have been transmitted to Japan along with the treasured poem.18

The Painting of the Nine Stages in the Nakamura Collection

I begin our examination of this genre and its remarkable transformation in Japanese culture with what I believe is the earliest type of the image. The handscroll of the nine stages in the Nakamura private collection, dated to the early fourteenth century (Fig. la-i, 12 5/8 by 195 1/8 inches, or 32 by 495.5 centimeters), is generally called the Kusoshi emaki (Illustrated Handscroll of the Poem of the Nine Stages of a Decaying Corpse; hereafter, the Nakamura version). The handscroll includes ten narrative illustrations, arranged from right to left. Before the depiction of the nine stages of decay, the sequence is prefaced by a portrait of a seated woman with long hair, in aristocratic attire, clearly a rendering of the painting’s subject before death (Fig. 2). Between her red lips, the white teeth covered by black pigment-a custom among aristocratic women-are visible.19 The predeath portrait suggests that the subject relished her beauty and wealth, a characterization expressed as well in the subsequent first stage of the newly deceased (Fig. 1a).20 In this stage, she lies with her head supported by a pillow on a raised tatami mat with ornamental trimmings. Her leaf-patterned undergarment covers most of her naked white body but leaves her right breast exposed, a distinctive feature of the Nakamura version.21 The first two illustrations seem to emphasize a sensual attractiveness springing from the woman’s voluptuous figure and noble background. In arousing an interest in the young beauty before delivering its lesson on taming desire, the image amplifies its cathartic value.

1 Illustrated Handscroll of the Poem of the Nine Stages of a Decaying Corpse, 14th century. Kanagawa, Nakamura Collection (photo: Tokyo National Museum). Stages one through nine, arranged right to left: (a) newly deceased; (b) distension; (c) rupture; (d) exudation of blood; (c) putrefaction; (f) discoloration and desiccation; (g) consumption by birds and animals; (h) skeleton; (i) disjointing.

In the third through tenth illustrations, a highly realistic process of decay unfolds in the scroll. The realism is cruelly accentuated in several ways. Each corpse looms large in its frame of 125/8 by 193/4 inches (32 by 50 centimeters), leaving little background, aggressively confronting the viewer with its image of corporeal decay. Given the scroll’s precise anatomical depictions, it has been suggested that this scroll follows the decomposition process of an actual, observed corpse.22 I agree with this view, and the illustrations in the sixth (Fig. 1f) and eighth stages (Fig. 1h) provide especially compelling visual evidence that there had been a model for these images. The painting of the sixth stage captures the network of sinews and muscles that appear under the parched skin. The complete skeleton in the eighth stage was drawn with a precise, confident brush. It is likely that the artist availed himself of the unburied corpses that were prevalent in Japan at this time.

The relation of the order of the nine stages in the Nakamura version to textual sources has been interpreted various-ly.23 The order of the stages of decay in the handscroll is as follows (see App. 1): (0) predeath portrait; (1) newly deceased; (2) distension; (3) rupture; (4) exudation of blood; (5) putrefaction; (6) discoloration and desiccation; (7) consumption by birds and animals; (8) skeleton; and (9) disjointing. The closest match of the order of decay in the Nakamura version is to the description of the Discourse on Mahayana Meditation and Contemplation, but with a few differences. An image of the newly deceased was inserted as the first stage of the Nakamura version, and to limit the total number to nine, the ninth stage of bones being parched to dust was omitted.24 Another, more significant, difference is found in the eighth stage. According to the text, in the stage of dismemberment, “the head and hands are located in different places, and five organs are detached from the body and shrunken.”25 The Nakamura version does not show the stage of dismemberment. Instead, it displays two different forms of bones in the eighth (Fig. 1h) and ninth stages (Fig. 1i): a whole skeleton and a disjointing of the bones. Thus, the key to interpreting the divergent order of decay in the Nakamura version lies in the reason for articulating two forms of the skeleton and omitting the stage of dismemberment.

As we have noted, descriptions of the nine stages of a decaying corpse are found in many Buddhist sources. The texts vary in their ordering and description of the decomposition process, but three share an identical sequence: the Discourse on Mahayana Meditation and Contemplation, the Discourse on the Great Wisdom, and the Explanations of the Doctrines on Meditation for Enlightenment (Japanese: Shakuzen haramitsu shidai homon, Chinese: Shichan bolomi cidi famen, by Zhiyi, 568-75). The lack of consistency among the surviving documents may indicate that the order itself was not critical. While the subtitles given to each of the nine stages vary among the sources, the designations share similar meanings for each of the relevant stages, with one obvious exception. Some sources specify one stage relating to bones, and others include two such stages: a whole skeleton and disjointed bones. For sources mentioning a single stage of bones, the texts refer to either a whole skeleton or disjointed bones. For sources that give contemplations on two stages of bones, the whole skeleton and the disjointed bones are designated as distinct objects for meditation in two sequential stages. In these sources, the stage of dismemberment is omitted to allow for the inclusion of two contemplations on different forms of bones. What could have motivated the distinct contemplations on a whole skeleton and the separated bones?

2 Predeath portrait of an aristocrat woman, from Illustrated Handscroll of the Poem of the Nine Stages of a Decaying Corpse, 14th century

Sequential contemplations on the whole skeleton and on the disjointed bones are found in six Buddhist textual sources: the Sutra of the Secrets for the Essential Way of Meditation (Japanese: Zenpiyohokyo, Chinese: Chan miyaofajing, translated by Kumarajiva, ca. 400), the Sutra of the Essentials of Meditation (Japanese: Zenyogyo, Chinese: Chanyaojing, translated before 220), the Chapters on the True. Meaning of Mahayana Teachings, the Su Tongpo and Kukai versions of the poems on a decaying corpse, and the Essentials of Salvation.26 None of these six texts includes a stage of dismemberment. Among them, the Sutra of the secrets for the Essential Wa-y of Meditation and the Sutra of the Essentials of Meditation are the oldest meditation manuals characterized by meticulous instructions in zen (Sanskrit: dhyana) practice.27 This zen meditation entails a specific sequence of concentrations on particular objects that help the practitioner to achieve a state of inner bliss. One of the methods for acquiring the transcendental state was contemplation on a decaying body.28

The Sutra of the secrets for the Essential Way of Meditation is one of the oldest texts for zen practice, and it no doubt influenced later meditative practices. In fact, the content of the sutra was incorporated into actual zen practices. Inside Caves 20 and 42 of the Toyuk Caves, Turfan, Xinjiang Province, China, mural paintings dated to between the mid-fifth and the seventh century29 show the substantial impact of the sutra.30 Both caves have extant images of monks contemplating on a decaying body and on a skeleton (Fig. 3).31 Thus, it is clear that from early times monks meditated on bones and (especially) on the whole skeleton as part of zen practice. The significance of the practice is reinforced by the emphasis given to the contemplation of the whole skeleton in o\ther meditation manuals.32

The two stages of skeletal bones also owe their provenance to the Discourse on Mahayana Meditation and Contemplation. The skeleton in the handscroll is depicted in pink, as if it had just lost its fleshy tissue. The pink hue (Fig. 1h) is clearly distinguished from the pure white color of the disjointed bones in the following stage (Fig. 1i). The distinction in color between the two stages closely resembles the description of the eighth stage found in the text: “Contemplate on the two kinds of bones: the one kind that is still covered by pus, and the other kind that is completely pure white. Or meditate on a set of bones, or their changing into disjointed fragments.”33

Further evidence that attests to the central role of the Discourse on Mahayana Meditation and Contemplation in the creation of the images in the Nakamura version is found in the zen practices as outlined in the text itself. Two different levels of contemplation on the nine stages are described. The lower level is contemplation on all stages up through the ninth, when the bones are parched to dust. The text notes,

3 A monk contemplating on a skeleton, Cave 42, Toyuk Caves, Turfan, ca. mid-5th-7th century (photo: courtesy of H. Sudo)

The practitioners who meditate in this way just seek to curtail their sufferings by trying to make the skeleton burn and disappear. They are in a rush to reach the fruit of arhatship [state of liberation], and are no longer enjoying the meditation on the phenomenal aspect of reality. Since they do not continue to contemplate the skeleton, they have no way to reach concentration, transcendental faculty, transformation, vows, wisdom, and the highest level of zen.34

The upper level of contemplation, by contrast, ends before the stage of bones parched to dust. The reason for this enigmatic truncation is now obvious, as contemplation on bones allows the practitioner to attain a transcendental level of meditation that brings the ultimate inner bliss. The Nakamura version follows the upper level of contemplation outlined in the discourse, as it ends with emphasis on the stage of the disjointed bones and does not depict the stage of bones parched to dust.35 Instead, a long blank space (13 inches, or 35 centimeters) is left at the end of the scroll after the last illustration, as if indicating that there is another stage in the text. Thus, we see that while the Nakamura version carefully follows the content of the Discourse on Mahayana Meditation and Contemplation, it was adjusted specifically to follow the upper level of contemplation for the utilitarian purpose of zen practice. No records have survived to verify the use of the Nakamura version itself, yet the scroll retains the pictorial elements of a prototype employed by monks in their ascetic meditative practices for overcoming sensual desires and, ultimately, for achieving a transcendental state. Let us consider the functions of the Nakamura version in detail.

The Nakamura version’s traditional title, Kusoshi emaki (Illustrated Handscroll of the Poem of the Nine Stages of a Decaying Corpse), is not original but was given to the work in 1977 based on scholarship that viewed the handscroll as an illustration of the Su Tongpo poem on the nine stages.36 Yet no texts of the poem are attached to the illustrations, and no companion handscroll containing the poem has survived, if it existed at all. I argue against this traditional view of the connection between the image and the poem because of the scroll’s transparent connection with the Discourse on Mahayana Meditation and Contemplation. In fact, on the lid of the wooden case for the Nakamura version, the inscription reads, “Kusozu (painting of the nine stages of a decaying corpse) painted by Tosa Mitsunobu [active ca. 1469-1523] at Saito [Western Pagoda], Jakkoin.” Jakkoin is a subsidiary temple of Enryakuji, the main temple of the Tendai school on Mount Hiei, in Shiga Prefecture,37 which early on placed an emphasis on the zen contemplative practice.

It is likely that the Tendai school’s early and deep association with zen meditation led to the creation of an image at Mount Hiei for ascetic practices that predated the fourteenth-century Nakamura version.38 This supposition is corroborated by an entry in the historical chronicle Mirror of the Eastern Court (Azuma kagami) for the eighth day of the eleventh month of 1212 (Kenryaku 2).39 The document recounts that a painting entitled the Flourish and Decay of the Life of Ono no Komachi (Ono no Komachi ichigo josui no koto) was shown at a picture competition held at the residence of the shogun Minamoto no Sanetomo, and it received the first-place prize. The painting of the ninth-century poet and legendary beauty is believed to have been an image of the nine stages of a decaying corpse. In fact, the Nakamura version was once identified as this work in a Tokyo National Museum exhibition catalog of 1974.40 A lack of strong evidence in support of the identification, however, resulted in the removal of this title for the painting. Nonetheless, the account suggests the existence of the earlier graphic depiction of the decaying corpse of a beauty, which could have been the model for the Nakamura version.

Later, the contemplation on the nine stages became associated with the Zen sect that focused on meditation practice. The biography of Muso Soseki (1275-1351), a prominent Zen monk, tells us that he used a painting of the nine stages at age fourteen in 1288.41 Thus, the image was known as a pictorial aid within the Zen monastic community. In addition, the two successive contemplations on the skeletal bones, an essential practice in the zen texts, are mentioned in the two apocryphal Kukai and Su Tongpo versions of the poems on the nine stages. Nakamura Hajime has suggested that the poems were composed by monks at the primary Zen temples in Kyoto called the Five Mountains.42 If true, the practice of two discrete contemplations on skeletal bones had been absorbed by the Zen monastic community. From its locus of origin in the Tendai school, the image of the nine stages began to spread beyond sectarian boundaries throughout medieval Japan.

After its involvement with Zen, the Tendai school at Mount Hiei went on to introduce Pure Land Buddhist belief to Japan. The first exponent of the new belief, which flourished from the late tenth century, was the Tendai monk Genshin, who wrote a seminal work on the Pure Land faith entitled the Essentials of Salvation. This treatise, which became the major work for the promulgation of Pure Land Buddhism in Japan, provided a new doctrinal and functional context for the image of the decaying corpse.

The Painting of the Nine Stages at Shoju Raigoji

Another early painting of the nine stages, dated to the late thirteenth century, is from a set of fifteen paintings entitled Six Realms of Reincarnation (Rokudoe) at Shoju Raigoji in Shiga Prefecture. Although this image is the earliest surviving work of the subject, I believe it represents an interpretation that postdates the type seen in the Nakamura version. A careful study of this work elucidates the entry of the theme into Pure Land Buddhist imagery and clarifies the functions of this example of the genre.

In order to understand the image, we need to take a brief plunge into Pure Land Buddhist cosmology. After death, living beings are thought to be reincarnated into one of six realms-hell, hungry ghosts, animals, titans, human beings, and divine beings-and they remain trapped in these realms if they fail to attain their rebirth in the Western Pure Land, the otherworldly place where the deceased reside with Amida Buddha. To give guidance to those who wish to be emancipated from the cycle of reincarnation, one chapter of Genshin’s Essentials of Salvation promotes a practice of contemplation on the horrifying aspects of the six realms. It is in the context of devotional contemplation on horror that our present image will find its locus of visual agency. The six realms of existence are treated in a set of paintings at the Shoju Raigoji, a Tendai Buddhist temple. The images complemented the agenda of the Essentials of Salvation, and in fact the scrolls have had a long association with the Pure Land Buddhist belief within the Tendai school. They have been housed in the Tendai temples at Reisan’in (from 1313 to 1538) and at Shoju Raigoji (from 1566 to the present).43 This version is found in a set of fifteen hanging scrolls with paintings that show selected scenes of pain, suffering, and torment from the six realms of reincarnation. Four of the fifteen scrolls treat the human realm and illustrate the aspects of existence: corporeal impurity, the suffering of birth and death, the suffering of war, and life’s transience. The scroll illustrating corporeal impurity is entitled Painting of the Impure Aspect of the Human Realm (jindo fujozu, Fig. 4, 611/4 by 25% inches, or 155.5 by 65 centimeters) and will be referenced hereafter as the Raigoji version.

It should be noted that the concept of the six realms of reincarnation was at first doctrinally independent from the contemplation on the nine stages. Nonetheless, the pictorialization of the stages of a decaying corpse was selected in the Raigqji version because it expressed the impurity of the human realm. The connection between the contemplation on the nine stages and the six realms of existence stems from the Essentials of Salvation,44 and while Genshin does not enumerate the nine stages themselves, the awful scenes of the six realms of existence in the Raigoji version refer to the content of this text. In fact, the inscriptions in the cartouches at the top of each scroll were taken from Genshin’s treatise.

In this image, which has suffered some fading over the last seven hundred years, the nine stages of a decaying female corpse are arranged in a zigzag fashion from the first illustration (newly deceased\, Fig. 5) at upper right to the ninth illustration (bones, Fig. 6) in the lower right corner, in the following order (see App. 1): (1) newly deceased; (2) distension; (3) rupture; (4) exudation of blood; (5) putrefaction; (6) discoloration and desiccation; (7) consumption by birds and animals; (8) dismemberment; and (9) bones. The order of decay after distension follows that of the Discourse on Mahayana Meditation and Contemplation, which influenced the Essentials of Salvation.45 The subject of the decaying corpse is itself lurid, and the images in this painting are rendered in a realistic and unabashed manner. Yet the pictorial details of the Raigoji painting fail to match either the natural process of human decomposition or the descriptions in the sutras. For example, it makes only a minimal visual distinction between the second stage of rupturing to the third of exuding blood, with a nearly identical body differentiated only by the casual application of red pigment in the third stage. The fifth stage of putrefaction (Fig. 7), according to the sutras, should depict the deformed corpse as if it were “wax melted by fire.”46 But in this image the corpse shows a desiccated state that is closer to the sixth stage (Fig. 8), when the color changes to bluish black through exposure to the wind and sun. In addition, the images of both the fourth and fifth stages, executed in sketchy brushstrokes, have a cartoonlike quality. The seventh stage, of consumption by birds and animals (Fig. 9), is marked by full, white flesh that mimics the illustration of the newly deceased, with no apparent recollection of the bluish black skin and desiccation of the previous stage.47 Thus, the Raigoji version captures the outline of the nine stages of a decaying female corpse and delivers a voyeuristic sensationalism derived from the pictorialization of the shocking motif, but it ignores textual and biological accuracy in the portrayal of the process of decay as described in doctrine, including the influential Discourse on Mahayana Meditation and Contemplation.

4 Painting of the Impure Aspect of the Human Realm, late 13th century. Shiga, Shoju Raigoji (photo: Nara National Museum)

5 Detail of Fig. 4: newly deceased

6 Detail of Fig. 4: bones

7 Detail of Fig. 4: putrefaction

8 Detail of Fig. 4: discoloration and desiccation

9 Detail of Fig. 4: consumption by birds and animals

The painting has a rectangular cartouche at the upper right corner with an inscription derived from Genshin’s Essentials of Salvation (Fig. 10):

The human realm has three aspects. One is the impure aspect. The body is largely filled with impurity. It is wrapped by seven layers of skin and nurtured by six tastes. But it is entirely odorous and defiled, and eventually putrefies from its attributes. If it is discarded between mounds, after one to seven days the body is swollen, the color is changed, and the skin is peeled. Before this aspect is seen, the attachment to affections is strong. But, if it is seen, all desires [for the body] cease.48

This inscription is not a verbatim quotation of the stages of a decaying corpse as they are described in the text; rather, it summarizes the entire section on the impure aspect of the human realm. The summary suggests that the painting was created not to focus on a step-by-step contemplation of the decomposition process but to convey the impurity of the human realm. A deeper knowledge of human defilement led to greater faith in Amida, as devotees sought escape from this world through rebirth in the Western Pure Land. Hence, the execution of the Raigoji version was in accord with the devotional purpose of the companion section of the Essentials of Salvation, and both works were created to inspire failli in Amida by describing the foulness of human existence.

The Raigoji version has a significant pictorial element-the landscape-that cannot be explained by religious texts. The hilly terrain in the Raigoji version is characterized by two noteworthy features. First, the soft contours of low hills are delineated with a minimum of textured strokes executed with a flat brush, the hallmarks of an archaic style of traditional Japanese painting called yamato-e, which attained its fullest development in the last quarter of the twelfth century. However, the attempt to create a sense of depth dates the painting to sometime about 1300. The landscape was colored mainly in dark ocher, with occasional areas in malachite green. Color tones between ocher and black (often with sparks of red), employed in a majority of the paintings of the six realms of transmigration, serve to convey the gloomy atmosphere of these defiled domains tainted by violence, illness, torture, misery, and a zoo of evils. The dark ocher in the Raigoji version provides a backdrop of barren, desolate ground on which the solitary corpse has been discarded, heightening the sense of man’s transitory existence. The second significant aspect of the landscape is the depiction of three trees, each a conventional emblem of its season, overhanging the corpse: cherry (spring), pine (summer), and maple (autumn). The cherry tree stands over the second stage of the distended corpse, the pine over the third of the ruptured corpse, and the maple over the fourth of the corpse exuding blood. The trees symbolize the passage of time and provide a metaphoric correspondence to the stages of the corpse’s decomposition.

10 Detail of Fig. 4: cartouche

11 Detail of Fig. 4: grave marker

The salient features of the landscape in the Raigoji version, desolation and seasonal trees, appear to be derived from Svi Tongpo’s poem of the contemplations on the nine stages of a decaying corpse (see App. 2).49 Although the poem was not written on the scroll, the painting seems to refer to its verses for the landscapes, probably because the Discourse on Mahayana Meditation and Contemplation gives no topographical descriptions.50 Consider, for example, the association between the seasonal trees and the Su Tongpo verses. The verse for the first stage eulogizes the complexion of the newly deceased:

Usual complexion paled during sickness. Fragrant body is as if sleeping. Beloved old friends still stay. The spirit has already departed. A beautiful face quickly fades as flowers in the third month. Life is brief like falling autumn leaves. No difference between youth and old age. No escape later or sooner, faster or slower.51

Here, the poem focuses on the appearance of the corpse, which hasjust started to discolor, likening it to “flowers in the third month [that is, early spring].” The painting itself shows cherry blossoms, the harbinger of spring, falling over the newly deceased body. No clear allusion to summer follows in the verses for the subsequent stages of decay, but the verses for the fifth and ninth stages contain terms related to autumn: shurin (long autumn rains) and akikaze (autumn wind). The painting shows the maple tree arranged directly over the fifth stage of the decaying corpse. The close correspondence evidences that the Raigoji painting was created by referring specifically to the Su Tongpo poem rather than the Kukai version, which includes no linear progression of seasonal changes during the process of decay.52

The other noteworthy feature of the landscape in the Raigoji painting, the desolate hills rendered mainly in ocher and some green, is also described in the Su Tongpo poem. For example, the verse for the second stage (distension) reads,

The distension of the newly deceased is hard to identify. After only seven days, mere vestiges of the [original] appearance remain. The rosy face has turned dark and lost its elegance. The raven hair, first withered, is now tangled with grass roots. Six organs are putrefied and the corpse pushes out beyond the coffin. The limbs have hardened and lie on the deserted field. The field is desolate, and no one is present. The spirit has gone to the other world in solitude.53

The motif of the desolate field or of green grasses beneath the corpse is repeated in every stanza of the poem, sometimes in the description of the accretion of tomb mounds.54 The Raigoji version follows the poem in its landscapes by placing each stage of the corpse’s decay in a barren, undulating field rendered in ocher with traces of green. At the fourth stage, the poem mentions the intermingling of old and new corpses beside the grave; indeed, the Raigoji version places the abandoned corpse beside the grave marker (Fig. 11) in the fourth image.

Thus, a correspondence emerges between the poetic descriptions of the Su Tongpo verses and the painted depictions of the Raigoji version. The correspondence is compelling in light of the fact that the companion Buddhist sutras offer no information about the landscape of this bleak scene. But the resonance of the poem in the painting goes beyond descriptive details. The Su Tongpo poem laments the transitory aspect (mujo) of this world and human life. The evocation of the changing seasons and the solitary corpse on the desolate field, integral elements of this theme, are employed in the painting as well. The notion of transience stems from Buddhism, but the sutras on the contemplation of the decaying corpse and human impurity make no explicit reference to the transitory nature of this world.55 Yet the concept of impermanence is suitable for a consideration of the cycle of life, death, and decay, and it infuses the exposition of the scenes in the Su Tongpo poem. In its landscape and portrayal of decay, the Raigoji version conveys the allusions in the Su Tongpo poem both to human impurity and to the impermanence of everything in the earthly realm.56 Thus, the Raigoji version may allow us to date the Su Tongpo poem to as early as 1300.

We now move to an exploration of the functions of this provocative image. The Raigoji version of the stages of a decaying corpse was one of a set of fifteen hanging scrolls whose content andinscriptions treat the six realms of reincarnation through reference to the Essentials of Salvation. Given this source, the significance of the image of the nine stages of a decaying corpse was substantially transformed by its treatment within the ambit of the six realms of existence. As we have noted, this text, authored by the Tendai monk Genshin, became the major work for the promulgation of Pure Land Buddhist belief. Genshin sought to inculcate Pure Land Buddhist belief by juxtaposing the blissful Western Pure Land with the pain and suffering of the six realms of existence, including the human realm and its characteristic impurity (represented by the nine stages of a decaying corpse). Furthermore, the Raigoji version encapsulated the descriptions of the Discourse on Mahayana Meditation and Contemplation, the framework of the Essentials of Salvation, and the pathos of the Su Tong poem. The devotional message was presented in a form suitable for public edification.

The inclusion of the nine stages of a decaying corpse in this visual juxtaposition seems to have begun about 1200. A medieval temple document, the New Essential Records of the Daigoji (Daigoji shinyoroku), records that the Enma Hall at Daigoji (Enma was the lord of the realm of the dead), commissioned by Senyomon’in (1181- 1252), the sixth daughter of Emperor Goshirakawa, and completed in the twelfth month of 1223 (Teio 2), displayed an image of the nine stages in its murals, which were lost when the building was destroyed in 1336.57 This textual record provides our earliest evidence for a depiction of the nine stages of a decaying corpse in the framework of the six realms of existence. Senyomon’in also commissioned an Amida Hall in the twelfth month of 1219 (Shokyu 1), and this structure was completed within the precincts of the same temple.58 Thus, Senyomon’in practiced her Pure Land Buddhist faith in the opposing (yet closely connected) spatial and spiritual domains of renunciation of the human realm in the Enma Hall and yearning for the paradise of the Western Pure Land in the Amida Hall, all in accord with the method employed by the Essentials of Salvation for deepening faith in the Buddha Amida.

The hanging scrolls portraying the six realms of reincarnation at Shoju Raigoji served as powerful visual agents for the exposition of Genshin’s doctrine. The practice of explaining religious beliefs through pictorial devices, called etoki (verbal explanation of pictures), began around the end of the twelfth century. While no documents have survived to verify that the set at Shoju Raigoji was employed in a didactic context, a record of the conservation dates of the paintings documents eight restorations between 1313 and 1683.59 The frequent restoration of the set may point to its use in public for etoki teaching. From the early twentieth century, the paintings have been displayed annually between the thirteenth and fifteenth days of the seventh lunar month as part of the annual ritual held to deliver ancestral spirits from the realms of suffering after death (umbon). In addition, the temple houses a script for etoki entitled the Abbreviated History of the Six Realms of Existence (Rokudoe soryaku engi) that explains the doctrines of the six realms with the use of paintings.60 The script was copied in 1897 at the request of a leading member of the temple, and while the date of the original is uncertain, it was likely transmitted at the temple for generations.

The earliest function of the image of the nine stages was for the pious contemplation on human impurity by Buddhist monks who wished to expunge the sensual desires that disturbed their lives of spiritual devotion. Therefore, the selection of a woman of exquisite beauty in the Nakamura version served to enhance the image’s original cathartic function of aiding male monks in their taming of sexual desire through viewing the stark opposition between comely beauty and repugnant decay. In fact, a major reference for early paintings of the nine stages of a decaying corpse, the Discourse on Mahayana Meditation and Contemplation, comments on the delusion caused by the beautiful appearance of an elegant woman and the effect of the contemplations on the nine stages for expelling sensual desires. The text admonishes,

Even a woman with graceful eyebrows, jadelike eyes, white teeth, and red lips is as if covered by a mixture of feces with fat powder, or as if a putrefied corpse were clothed with silk and twill. … a contemplation like this [on the impurity of a decaying corpse] is a golden remedy for sensual desire.61

Other earlier Buddhist sutras mention that the contemplation on a corpse is effective for curbing sensual desires, but they make no reference to the gender or appearance of the corpse.62

The selection of a beautiful, aristocratic woman was linked to the expression of transience in the Raigoji version. Such an association is supported by the aforementioned entry in the historical chronicle Mirror of the Eastern Court for the eighth day of the eleventh month of 1212 (Kenryaku 2).63 This document recounted that a painting entitled the Flourish and Decay of the Life of Ono no Komachi was shown at a picture competition held at the residence of the shogun Minamoto no Sanetomo.64 The title assigned to the painting of the nine stages of a decaying corpse, “the flourish and decay,” stems from a deep-seated medieval notion of transience, in particular as experienced in the lives and fortunes of women. The transient aspects of women’s lives form a theme in the medieval literary works of female authors (including the legendary Ono no Komachi), a theme fundamentally rooted in their tragic love affairs in the polygamous society.65 According to Buddhist doctrine, five obstacles to enlightenment and three kinds of required obedience (to parents, husband, and children, after the husband’s death) shaped the woman’s lamentable lot.66 The characterization of the decaying corpse as a beautiful, aristocratic female-an image that conveyed the epitome of human transience-was important to the new function of the nine stages in the Raigoji version. In the painting, the two Buddhist notions of human impurity and transience were subtly and overtly integrated for didactic impact in the exhortation of Pure Land Buddhist belief.

I should point out here that the reference in the Mirror of the Eastern Court to a painting of Ono no Komachi has led to frequent misidentifications of the female corpse in paintings of the nine stages, including the Raigoji version, as the ninthcentury figure herself. Ono no Komachi was celebrated for her poetic talent, her stunning beauty during her youth, her trifling with amorous men, and her suffering from decrepitude and destitution in old age. The earliest tale mythologizing the poet is found in the Flourish and Decay of the Life of Tamatsukuri no Komachi (Tamatsukun no Komachi sosuisho), dated perhaps about 1200. Later, the popularity of Ono no Komachi increased as she became the central subject of five Noh plays, among which Sotoba Komachi (written by Kan’ami [1333-1384] or Zeami [1363-1443]) captures her hardship in her old age.67 In the eighteenth and nineteenth centuries, some paintings of the nine stages of a decaying corpse were given the title The Nine Stages of Ono no Komachi’s Decaying Corpse. After this identification of the female corpse as Ono no Komachi became established in the Edo period, the cadaver in antecedent versions of the painting of the nine stages, including the Raigoji version, has been incorrectly and anachronistically regarded by some as a biographical image of the ninth-century poet.68 Yet it is unlikely that a Buddhist devotional image would center on a specific poet and that the tragic, secular female figure Ono no Komachi could have been portrayed in a painting produced by a temple. It is more reasonable to posit that the corpse was an anonymous paragon of beauty and decay. The inaccurate appellation probably arose from a desire among general audiences to establish an identity for the woman in the startling image. Indeed, over the centuries, the beautiful aristocrat of the Raigoji version has also been connected with other legendary beauties. For example, some later paintings of the nine stages were thought to represent Empress Danrin (Emperor Saga’s wife, 786-850). The Abbreviated History of the Six Realms of Existence, dated to the nineteenth century, comments on the female corpse of the Raigoji version:

The woman in this [Raigoji] painting is either Empress Komyo or Empress Danrin. These two empresses were exceptionally beautiful during their lifetimes, and every man adored them at first sight. They stipulated in their wills that after the moment of death, their bodies should be discarded on the field of the Western Hill. Everybody, rich and poor, man and woman, crowded at the market in order to see their corpses. What they saw was the gradual process of the corpses’ decay to white bones. These two empresses exposed their corpses to the public with the hope that, since all will be equally impure after death, sentient beings in the Latter Days of the Buddhist Law should be awakened through exposure to the impure human condition.69

There is no historical evidence to substantiate this anecdote, but it seems to be another instance in which the identity of the female corpse was misrepresented in order to connect the image with a legendary beauty. Such attempts to associate the corpse with historical figures peaked in the eighteenth and nineteenth centuries. They arose from a popular literary genre teaching the ideal way of female life that thrived between the mid-sixteenth and the late seventeenth centuries.70 (This issue will be examined presently.)

It may be observed that the portrayal of a woman in the Raigoji version had an ancillary benefit for the promulgation of Pure Land Buddhism. In traditional Buddhist teaching, \women were viewed as impure and inferior to men. Women rarely attained salvation, even with extreme devotion, unless they were transformed into men at the moment of death. But Pure Land Buddhist doctrine, remarkably, promises that women could gain salvation as women. While the doctrinal innovation is already evident in a significant Pure Land Buddhist text, the Larger Sutra (Japanese: Muryojukyo, Sanskrit: Sukhovati-vyuha, translated by Buddhabhadra and Baoyun [375P-449], 421), its full import had to await Honen’s (11331212) commentary on the sutra, dated 1190.71 Allowing the salvation of women made it possible for Pure Land Buddhism to attract devotees of both sexes. The Raigoji version, which was created just after the completion of Honen’s highly influential commentary, might also have inspired women to pursue rebirth in the Western Pure Land. Since this version of the nine stages was intended as an image for public instruction, its audience included both men and women. The doctrinal openness of the image to both genders signaled a marked shift from the exclusively male audience of Buddhist monks targeted by the earlier type of the Nakamura version.

At this juncture we can consider the way in which Pure Land Buddhism encouraged the spread of this provocative image of decay. Although the prototype of the Nakamura version probably existed earlier, there are no textual records mentioning images of the nine stages of a decaying corpse in Japan before about 1200. The dearth of references to the images indicates that the nine stages of a decaying corpse were not subject to popular use before the absorption of Pure Land Buddhism. What may have hindered broader interest in this theme? In medieval Japan, there were deep-seated beliefs about defilement from particular objects, incidents, and conditions. We know from medieval records and diaries that such threats to purity were carefully categorized and rules given for their expurgation. In these regulations, death and the dead body figured among the sources of defilement cited most frequently.72 Not only was the corpse seen as unclean, but the defilement was also considered contagious. It is unclear how a painting of a corpse would have been treated in light of these beliefs, but without an overriding religious motivation, such an image would not have been produced in Japan. Breaking the indigenous taboos and encouraging people to face a corpse for the sake of devotion required a new theological foundation. In this sense, Pure Land Buddhist belief provided the basis that made possible a focused contemplation on death and the corpse.’3 Unlike earlier schools of Buddhism that restricted a blissful afterlife to the few of the religious elite who succeeded in achieving liberation from vicious transmigratory cycles, Pure Land Buddhism presented a new manner of devotion to Buddha Amida that could be managed by even lay devotees, thus opening salvation to any who practiced simple nenbutsu (to think of the Buddha).74 In other words, devotees could now encounter death and corpses, previously untouchable, knowing that their proper devotion to Buddha Amida would assure them of rebirth in the Western Pure Land. Consequently, the image of the nine stages of a decaying corpse was created and circulated contemporaneously with the culmination of Pure Land Buddhism in medieval Japan.

12 Illustrated Handscroll of the Poem of the Nine Stages of a Derating Corpse, 1527. Osaka, Dainenbutsuji (photo: Osaka Museum of History). Stages one through nine, arranged right to left, with intervening poems excised: (a) newly deceased; (b) distension; (c) exudation of blood; (d) putrefaction; (e) discoloration and desiccation; (f) consumption by birds and animals; (g) whole skeleton; (h) disjointing; (i) parched to dust.

The Painting of the Nine Stages at Dainenbutsuji

Images of the nine stages of a decaying corpse were produced through the nineteenth century. While these retained the sensational subject, they had entirely different functions from those of the early paintings. The examination of two later works will elucidate the transformation of the image within distinct religious and cultural contexts. We turn first to the image of the nine stages at Dainenbutsuji, Osaka (Fig. 12a-i, 1214 by 1841/2 inches, or 30.8 by 468.6 centimeters; hereafter, the Dainenbutsuji version). The Dainenbutsuji is the head temple of the Yuzu (all-inclusive) Nenbutsu school, founded by the priest Ryonin (1072-1132). According to an inscription at the end of the handscroll, the Dainenbutsuji version was created in 1527 (Daiei 7).75 The scroll begins with a picture of the crescent moon and autumn grasses painted in silver and gold pigments.76 Next is inserted a section containing the wavy watermark decoration that often accompanies calligraphic verses, followed by the preface to the Su Tongpo poem (Fig. 13). Each stage of decay is then presented, with the relevant stanza of the poem, written in Chinese characters, and the usual two waka, inscribed in a mixture of Chinese characters and Japanese kana syllabary. The calligraphy of the preface and poems is executed with gilt decorations that include seasonal plants, landscape, and birds. The Dainenbutsuji version is the earliest surviving painting of the nine stages to be accompanied by both the Su Tongpo poem and the waka verses. Through a graphological analysis of the poems, the writer has been identified as a prominent aristocratic monk, Johoji Kojo (1453-1538), who was renowned for his skillful calligraphy.77 No records regarding the provenance of the handscroll have survived, but the painting is likely to have been located at Dainenbutsuji since the early sixteenth century. The calligraphier Johoji Kojo was once an abbot of the Kuramadera temple, which had a long association with the Dainenbutsuji’s founder, Ryonin, and it is likely that Johoji would have joined the project at the Dainenbutsuji. The painter is unknown, but the work is attributed to the studio of Kano Motonobu (1476-1558), the second-generation head of the famed Kano school of painters. As prominent artists were involved in the creation of the Dainenbutsuji version, we may assume that an affluent patron must have commissioned the work.

13 Initial scene and preface of the Su Tongpo poem, from Illustrated Handscroll of the Poem of the Nine Stages of a Decaying Corpse, 1527 (photo: Osaka Museum of History)

Each stage in this version is placed after the relevant subtitle from right to left (see App. 1): (1) newly deceased (shinshiso); (2) distension (hochoso); (3) exudation of blood (ketsuzuso); (4) putrefaction (horanso); (5) discoloration and desiccation (seioso); (6) consumption by birds and animals (shokutanso); (7) whole skeleton (hakkotsurenso); (8) disjointing (hakltotsusanso); (9) parched to dust (jokeso). One distinctive feature of the Dainenbutsuji version is its visual correspondence to the accompanying Su Tongpo poem. The nine images have landscapes delineating the graveyard where the dead body has been discarded, and some of the landscape motifs are derived from the poem. For example, in the first stage of the newly deceased (Fig. 12a), both the seasonal cherry and maple trees are painted near the corpse in an echo of the verse: “A beautiful face quickly fades as flowers in the third month. Life is brief like falling autumn leaves.” In the fifth stage (Fig. 12e), the painting follows the poem by showing the morning sun drying out the corpse. The weeds and the pine tree in the eighth (Fig. 12h) and ninth (Fig. 12i) stages echo the poem as well. Yet visual elements that impede the flow of the narration as well as the poem’s conveyance of seasonal change preclude a close coordination of the pictorial images and poetic motifs. The first stage, with cherry and maple, simultaneously connotes spring and fall. The autumnal scenes, in the second through fifth stages, and the wintry scenes, in the six through eighth stages, are concluded with summer scenery (denoted by the morning glories) in the last frame. In addition, the particular area of the graveyard rendered in the painting does not remain constant throughout the nine scenes, altered by the casual addition of motifs corresponding to the poem and by changes in major landmarks (such as mountains, trees, and rocks) that convey different locations. Because of the inconsistency of the landscapes throughout the sequence, the process of the corpse’s decay plays the primary narrative role. As we have noted, later renderings of the nine stages, including the Dainenbutsuji version, were often attended by both the Su Tongpo poem and the waka.78 The two waka written near each of the nine stages of the Dainenbutsuji version rarely pertain to the stage of the corpse that they accompany.79 The poems capture only the general atmosphere of the pathos of transience underlying the decay of the corpse. The authorship and date of the waka are uncertain, but we do know that the verses were circulated in the late fourteenth century.

The artistic style of this version warrants special attention. The depiction of the corpse lacks both anatomical precision and a meticulous observation of the process of decay. The eight corpses lying in changing settings look like dolls propped up to mark only the essential plot. The sensational subject is softened further by the yamato-e landscapes with the skillful use of hovering fog (suyari gasumi), a traditional pictorial device of this style that unifies the scenes of the sequence over the passage of time.80 The visual depiction of the Dainenbutsuji version concludes by showing a male aristocrat weeping in front of four stupas (sotoba, containers for relics or symbolic sacred objects)-an addition unique to this handscroll (Fig. 12i).81

14 Pious wife of a lower-ranked monk being returned from the realm of dead to this world, from Legends of the Yuzu Nenbutsu: Yuzu Nenbutsu Engi, handscroll, \ink, color, and gold on paper, 35 X 485 in. (89.7 X 1232.4 cm), 14th century. The Cleveland Museum of Art, Mr. and Mrs. William H. Marlatt Fund, John L. Severance Fund, Edward L. Whittemore Fund (photo: The Cleveland Museum of Art)

The early images of the nine stages of a decaying corpse are powerfully didactic in their graphic impact. Their visual effectiveness stems from their exposition of human impurity or their connection to the concept of the six realms of reincarnation. In the Dainenbutsuji version, in contrast, the shocking details are no longer articulated. The illustrations have been sanitized from the grotesque instructive descriptions of the decaying corpse in a preference for reflecting the atmosphere and selected elements of the accompanying poems. The simplified illustrations of the corpses in the Dainenbutsuji version even seem to laugh rather than howl in death, perhaps out of an intention to deliver the subject of the decaying corpse in a less hortative manner.

Let us now consider the substantial transformation of the image of the nine stages between about 1300 and 1527 from the standpoint of its functions and historical background. The function of the Dainenbutsuji version is illuminated by a genre of Buddhist narrative found at the same temple. As the seat of the Yuzu Nenbutsu school, the Dainenbutsuji temple emphasized the idea of yuzu, melding different substances together, their union bringing perfection through synergism. The coalescence of faith resulting from chanting nenbutsu with other devotees was thought to eventually bring the practitioners to rebirth in the Western Pure Land. It was said that the school’s founder, Ryonin, in order to amplify the synergistic effect encouraged all devotees to recite nenbutsu ten times every morning facing west as a mass thaumaturgie practice. The faith was popular among Buddhists regardless of sectarian affiliation, but it was not until 1661 that the yuzu nenbutsu belief became an official Buddhist school.82

Art historically, the school is best known for the Yuzu nenbutsu engi emaki (Illustrated Handscrolls of the Legends of Yuzu Nenbutsu; hereafter, the Yuzu emaki). Since the production of the first two volumes of the handscroll in 1314, the same format and subject have been painted repeatedly.83 In illustrations and calligraphic texts, the first volume narrates the life of Ryonin, while the second volume depicts the auspicious and miraculous events that befell the practitioners of yuzu nenbutsu (Fig. 14). Twenty-eight versions of the handscroll have survived, most of them dated between the fourteenth and fifteenth centuries, except for several nineteenthcentury copies of the earlier versions. The creation of so many versions of an illustrated handscroll having the same subject is exceptional in Japanese art history, and their extensive production reveals the distinct function of the handscrolls within yuzu nenbutsu belief.

The characteristic teaching of the school was that the spiritual practice of a single person results in merit for all, and therefore the devotional actions of a multitude increase the salvific benefit exponentially. The religious duties of yuzu nenbutsu included the offering of oblations to itinerant monks who preached the belief. Since yuzu nenbutsu practice was nonsectarian, the solicitation of offerings was not confined to monks of Ryonin’s lineage. However, the creation of the Yuzu emaki for the purposes of outreach and solicitation was undertaken largely by Ryonin’s successors. The illustrated handscrolls that explained the miraculous events of Ryonin’s life and of the yuzu nenbutsu practitioners served as a way of legitimizing the spiritual efficacy of the belief and attracting new devotees.84 The Yuzu emaki became visual aids for the aggressive exposition of the sect’s teachings to broad strata of society, from which it could collect contributions and donations. Such a missionary scheme was typically utilized for the illustrated handscrolls at Shinto shrines and Buddhist temples that were widely produced from the thirteenth century on; indeed, the handscroll became a significant medium for garnering capital, particularly under the Ashikaga military government (1336-1571), when Buddhist temples, which had prospered under the estate system of landholding backed by the authority of the central government, could expect little financial support from the weakened court.

A representative preface of one Yuzu emaki reveals this aim:

The monks and laity, who heard about the miraculous power and the fortunate examples, wished for the same. If they make bonds [through yuzu nenbutsu teaching] by practicing nenbutsu and writing their names, they will be free from all misfortune during this life, and they will attain their desire for salvation in the next life. . . . The intention for painting the teaching of the yuzu nenbutsu is to foster belief among the male and female laity.85

We see here that the goal of the handscrolls, from the standpoint of the temple, was to create and strengthen ties between the temple and its financially supportive devotees. The content of the Yuzu emaki centered on two kinds of stories. One type recounts how the yuzu nenbutsu was expounded to many people, rich and poor, male and female, clergy and laity, all of whom attained salvation through nenbutsu practice. Stories of the other type tell how practitioners were protected by Buddhist deities, such as Amida and Bishamonten (Sanskrit: Vaisravana), and they relate the ways in which the power of yuzu nenbutsu wrought miracles, such as the revival of a monk’s wife from the realm of the dead (Fig. 14) and the recovery of a cowherd’s wife from a difficult delivery. It is worth noting that this latter type of story emphasized the miracles that befell female devotees, and such a predilection reflected the school’s desire to broadcast the new faith to all.

The compact format of the Yuzu emaki handscrolls was handy for the itinerant priests who urged people to perform meritorious acts, including making donations to temples.86 In their travels, these priests would preach the content of

A Shot in the Arm for Women ; Growing Number of Female Doctors Alters Medical Field

Agnessa Gadeliya’s idealism drew her to medicine, along with the mental stimulation she derives from diagnosing diseases.

Gadeliya also sees a practical benefit to becoming a physician: It has increasingly become an attractive career for someone who wants to balance home and family life.

“If I ever have kids, of course I’ll work part time,” said Gadeliya, a third-year medical student at the University of Colorado School of Medicine.

In 2003, the medical profession turned a corner. The Washington, D.C.-based Association of American Medical Colleges reported that for the first time more women than men applied for admission to U.S. medical schools.

The following year, the percent of female applicants held steady at 50.4 percent. At the University of Colorado, female applicants outnumbered males in 2000 and 2001 but fell slightly below half in 2002, 2003 and 2004.

Women make up 20 percent to 30 percent of all doctors. Their presence has influenced the practice of medicine, the structure of work and salaries.

“It’s a wonderful career for women because there is so much diversity in what you can do,” said Maureen Garrity, the CU School of Medicine’s associate dean for medical student admissions. That opinion defies traditional notions of medicine as an all-consuming calling.

“Women are starting to figure out you can do it and still have families and a personal life.”

Women surgeons scarce

J.J. Stroh grew up as the daughter of a Colorado Springs veterinarian and views her future job in family medicine in the tradition of lifelong relationships.

“You’re taking care of the entire family, from birth to old age,” said the 30-year-old family medicine resident.

Stroh is typical of female physicians. According to the Association of American Medical Colleges, females outnumber or equal male residents in less procedure-oriented, more nurturing primary care specialties such as pediatrics, psychiatry and family medicine.

Women also are drawn toward dermatology and emergency medicine because set hours in those specialties allow for a personal life. Obstetrics and gynecology also are popular with women because many patients demand a female doctor. But women remain scarce in other surgical fields.

Danielle Haakinson, 28, a first-year medical student, plans on a career in emergency medicine “because it is sort of shift work,” she said.

In 2004, 68 percent of pediatric residents were female. Women made up 51 percent of family medicine residents and 41 percent of internal medicine residents. Just 12 percent of neurosurgery residents and 5 percent of interventional cardiology residents were women.

Experts say resident work hours and a lack of mentors influence the gender trend. Surgical residencies demand a grueling 80-hour workweek and are timed just as female residents may want to start families.

“(Surgical specialties) have been slow to change their style and expectations of members around work hours and scheduling,” said Dr. Colleen Conry, vice chair of the family medicine department at CU. “It still is more of an old-boy culture.”

Conry is grateful the early days of women in medicine are over. When she started her studies 25 years ago, a Kansas City taxi cab driver refused to take her to medical school.

“He said, ‘You want the school of nursing,’ ” she recalls. “I said, ‘No, I want the school of medicine.’ He dropped me off at the school of nursing.”

Today, the medical industry has come to value what traditionally are regarded as female qualities of empathy and good listening, experts say.

Research shows female doctors spend more time with patients, ask more personal questions and do a better job getting patients to agree to treatment.

No studies exist, but malpractice experts believe women get sued less as a result.

“We think that communication has something to do with your likelihood of having a lawsuit in the event of a bad outcome,” said Dr. Richard Quinn, a physician risk manager with COPIC Insurance Co. “It’s sort of self-evident. The nature of the female in dealing with traumatic events is more of a nurturing type of approach, and we certainly recommend it.”

Part-time trend causes alarm

A shift in medicine over the last few decades from private to group practice, including health maintenance organizations, has aided women in medicine. Group practice enables doctors to share responsibilities and have more opportunities to work part time or to otherwise arrange family-friendly schedules.

Kaiser Permanente employs 267 female doctors, and 61 percent work part time. Of Kaiser’s 440 male physician employees, 18 percent work part time.

“We totally support the life-work balance,” said Jacque Murphy Montgomery, a Kaiser spokeswoman. “It’s actually a recruiting strategy.”

Yet the popularity of part-time work is causing some alarm. Various studies have pointed to a physician shortage developing over the next 20 years, with the growth of part-time workers as one cause.

“Apples aren’t replacing apples as doctors are retiring,” said Kurt Mosley, a vice president of business development for the Irving, Texas-based MHA Group, a health care staffing company. “We have to have 11/2 or maybe two females to replace one retiring physician.”

Nonetheless, the physician shortage allows doctors to enjoy plenty of job opportunities.

While medicine isn’t the path to wealth that it once was, it remains a lucrative field.

According to an annual survey by the American Medical Group Association, in 2004 emergency medicine doctors earned $227,000 a year; family practice doctors, $166,000; and radiologists, $412,000.

Younger doctors seem less concerned about pay than previous generations did.

“I’m really not doing it for the money,” said Stroh, the family practice resident. “If I was, I would have picked another speciality.”

Despite advances in medical school applications, women continue to lag on medical school faculties.

According to the medical colleges association, only 15 percent of medical school faculty at the rank of full professor and 10 percent of deans are women.

A study at the University of Arizona found that female faculty members consistently earn about 11 percent less than their male counterparts, even after clinical productivity and research hours are factored in.

At the University of Colorado, just two out of 24 department chairs are women, and just 13 division and section heads – out of 56 divisions and sections – are women.

It takes 14 years to become a full professor, and some experts say women are just catching up.

“I still think the number of women in leadership positions is lower than what you would expect, given the denominator,” said Dr. Jean Kutner, division head of internal medicine at the University of Colorado, whose department includes more than 100 doctors who report to her.

“Because there are few women in leadership positions, there are few role models. There’re not many people out there where you’d say, ‘Yep, that’s the person I want to be when I grow up.’ “

Outside of academic medicine, the last bastion of resistance to young female doctors may be patients.

Stroh, doing a residency in Greeley, recalls more than once outlining a care plan to her patients.

“That’s fine,” patients would say, “but when is the real doctor coming in?”

“That would be me,” she replies.

INFOBOX

Medical specialties by pay and gender

Highest-paid specialties Pay Percent female

Neurosurgery $421,000 5 percent

Cardiac, thoracic surgery $417,000 3 percent

Diagnostic radiology $412,000 21 percent

Orthopedic surgery $354,000 4 percent Lowest-paid specialties Pay Percent female

Internal medicine $172,000 30 percent

Pediatrics $172,000 51 percent

Family practice $166,000 32 percent

Sources: American Medical Group Association; The American Medical Association

Gastrointestinal Neurofibromatosis: An Unusual Cause of Gastric Outlet Obstruction

Neurofibromatosis type-1 (NF-1), also known as von Recklinghausen disease, is a common autosomal dominant condition occurring in approximately 1/3000 births. NF-1 is known to be associated with gastrointestinal neoplasms in 2-25 per cent of patients. We report the first case of gastric outlet obstruction with perforation caused by neurofibroma in a patient with NF-1. The literature is reviewed, examining 61 previously reported cases of noncarcinoid gastrointestinal (GI) neoplasms in patients with NF-1 for symptoms, location, and types of neoplasms. Neoplasms were located most often in the small intestine (72%). Neurofibromas, found in 52 per cent of patients, were the most frequently diagnosed benign neoplasms followed by leiomyomas (13%), ganglioneurofibromas (9.8%), and gastrointestinal stomal tumor (GIST) (6.5%). Adenocarcinoma was present in 23 per cent of patients. Patients with NF-1 and GI symptoms are at risk for gastrointestinal neoplasms from which symptomatic patients are likely to experience significant morbidity.

NEUROFIBROMATOSIS TYPE-1 (NF-1), also known as von Recklinghausen neurofibromatosis, is a common autosomal dominant condition occurring in approximately 1/3000 births.1-3 NF-1 is known to be associated with gastrointestinal neoplasms in 2-25 per cent of patients.3-6

Case Report

A 21-year-old African-American man presented to the hospital emergency department with a chief complaint of gastrointestinal (GI) bleed. He arrived with periumbilical pain, intermittent nausea, vomiting, and anorexia gradually worsening over 3 to 4 days, now with transitory coffee-ground emesis. He reported no bowel movement for 3 days. He denied fever, hematochezia, or dysuria. His past medical history was significant for NF-1 complicated by a below- knee amputation during childhood, related to his NF-1, and two episodes of GI bleed: the first, 5 years prior to presentation, and the second episode, 8 weeks prior to presentation, was treated with H-2 blocker.

On physical exam, the patient was awake, alert, and oriented. His mucous membranes were dry. His abdomen was taut, distended, diffusely tender with guarding and rebound tenderness. Bowel sounds were hypoactive. The patient’s physical condition declined while under observation. His temperature increased from 38.1C to 38.9C, blood pressure of 100/88 mm Hg dropped to 100/80 mm Hg, and heart rate increased from 120-130 heats per minute with respirations of 18 per minute. Blood chemistry, amylase, and lipase remained within normal limits. Coagulation studies were abnormal with an elevated international normalized ratio (INR) at 1.4 (normal. 0.8-1.0). Stool was heme negative.

Free air, multiple air fluid levels, and small bowel distention were seen on X-ray obstruction series (Fig. 1). These findings led to the working diagnosis of perforated viscous. While undergoing fluid resuscitation, the patient was transported from the emergency department to the operating room with an acute abdomen.

At exploratory laparotomy, a perforated 3.5- to 4-cm ulcer located on the anterior surface of the mid portion of the stomach was seen. The stomach appeared thick-walled, however, no mass lesions were noted. The omentum was closely approximated to the inflammation and formed a phlegmon extending to the posterior aspect of the stomach and pancreatic bed involving the lesser sac. Multiple enlarged lymph nodes were present. The patient underwent a subtotal gastrectomy with a Billroth II gastrojejunostomy for a perforated gastric ulcer.

Gross examination of the antrectomy specimen revealed an 8.5 cm by 7 cm by 5 cm specimen with a 2-cm transmural perforating ulcer seen on the anterior wall of the stomach (Fig. 2). The gastric wall appeared thickened throughout with stenosis at the gastroduodenal junction. However, no discrete mucosal lesions were identified aside from the perforation.

On microscopic examination, diffuse neurofibromatosis was present, predominantly localized to the submucosa but involving all layers of the gastric wall. In addition, plexiform neurofibromas were seen associated with the areas of diffuse neurofibromatosis and extending into the subserosal fat. A decreased number of oxyntic cells were noted. Throughout the body end of the specimen, vascular changes of marked intimai hypertrophy, media hypertrophy, and occasional recunali/ed thrombi were noted in areas where neurofibroma had elicited collagenous reaction. No vasculitis was present.

FIG. 1. Chest X-ray: Lateral view demonstrating free air seen just below the diaphragm.

FIG. 2. Gross pathology demonstrating gastric perforation surrounded by erosive gastritis.

The area of perforation showed granulation tissue with a prominent reactive fibroproliferation. Within the segment of duodenum, multiple intramuscular neurofibromas extended into the submucosa and were associated with Brunner’s gland hyperplasia. The neurofibroma was diffuse and diminished the lumen of the duodenum (Fig. 3). A ganglioneuromatous proliferation was seen to replace the lamina propria focally. No neoplasm, necrosis, significant mitotic activity, or changes suggestive of malignant transformation were seen. There was no evidence of Helicohacter pylori, and the pacemaker cells of Cajal were normal in number.

The patient’s postoperative course was complicated by bowel obstruction caused by extensive adhesions. Pathologic examination of additionally resected small bowel showed Auerbach plexus hyperplasia in addition to the adhesions. No neurofibromas were seen.

Discussion

This case represents the first reported case of NF-1 and gastric outlet obstruction caused by neurofibromas resulting in perforation. Peptic ulcer, neoplasia, benign tumors, inflammation, and rare miscellaneous causes are known causes of gastric outlet obstruction. Malignancy must be excluded in all cases, as it can be present in 50 per cent of cases.

FIG. 3. Low power demonstrating the first portion of the duodenum with muscular hypertrophy and diffuse submucosal neurofibromas narrowing the lumen, causing gastric outlet obstruction.

An English literature search of gastrointestinal neoplasms and NF- 1 from 1975 to 2002 resulted in 61 previously reported cases associated with neoplasms of the GI tract.4, 6-63 Cases were excluded for carcinoid, pheochromocytoma, or other concurrent illnesses such as polyposis syndromes or birth defects.

No gastric perforations have been reported, however, three reported cases of intestinal perforation associated with NF-1 have been published.56, 59, 60 This makes perforation either a rare complication of a rare condition (GI-NF-1) or underreported. All reported perforations involved benign neoplasms located in the jejunum (Table 1). The site is likely reflective of the increased incidence of neoplasia at this location.

Due to its rarity, a high degree of suspicion for gastrointestinal tumor in patients with NF-1 is needed for timely diagnosis. Delay in diagnosis of gastrointestinal neurofibromatosis is common. The average interval from onset of GI symptoms to diagnosis with GI neoplasm was 2.8 years in patients reviewed in this study. Diagnosis may be difficult due to nonspecific symptoms, as well the predominantly small bowel location. Neurofibromas in particular may be diffuse and submucosal, making radiographic visualization difficult.

The development of neoplasms should be considered to be part of the disease process in patients with neurofibromatosis type-1.64 The gene for neurofibromatosis type-1 has been localized to chromosome 17 and encodes an RNA product called neurofibromin. Neurofibromin acts as a neoplasm suppressor,65 and patients with NF-1 have decreased levels of neurofibromin. This may explain the increased incidence of malignant neoplasms of all types in patients with neurofibromatosis compared to the general population.64

Among all 61 patients with neurofibromatosis and gastrointestinal neoplasm of any type, our patient was younger than the average age of 46 for diagnosis with GI neoplasm. Sixty-nine per cent of patients were over age 40 at time of diagnosis. More males, 59 per cent (n = 36), than females, 41 per cent (n = 25), were represented.

Symptoms of GI bleed (bright red blood per rectum), heme + stool, or hemetemesis were exhibited by 46 per cent (n = 28) of all patients. GI obstructive symptoms (obstruction, abdominal distention, and constipation with vomiting) were present in 16 per cent (n = 10) of patients. Hepatobiliary symptoms of jaundice or biliary obstruction were reported in 16 per cent (n = 10). Hepatobiliary symptoms were not found under age 20 and were associated with stomach or duodenal neoplasm in 8 of 10 cases and adenocarcinoma of the duodenum in 6 of 10 cases. Fifteen patients had only nonspecific gastrointestinal symptoms.

TABLE 1. Reported Cases in Patients With Neurofibromatosis, Noncarcinoid Neoplasm, and Perforation Since 1975

The most frequent location of GI neoplasms was the small intestine where 72 per cent (n = 44) of patients had neoplasms (Table 2). Most of these were found in the jejunum, 39.3 percent (n = 24). Forty-one percent (n = 25) of 61 patients had neoplasms in multiple locations.

The large percentage of small intestine tumors found in this population is in contr\ast to the general population in which small bowel neoplasms are rare, occurring at this location in only 5 per cent of cases.66 Comparison between these two populations also reveals that for patients with neurofibromatosis, neoplasms occur at a younger age (46 years compared to 59 years) and are most frequently neurofibromas, whereas leiomyomas are the most frequent benign small bowel neoplasm in the general population. In a patient with neurofibromatosis and GI symptoms, a high degree of suspicion for small bowel neoplasms should be maintained, although the reason for increased incidence of neoplasms at this location remains elusive.

Most patients with GI neoplasms and neurofibromatosis have benign neoplasms (Table 3). Benign neurofibromas are the most frequently occurring GI neoplasm for patients with NF-1. Auerbach plexus hyperplasia, as was noted in our patient, may be their site of origin. Benign neurofibromas undergo malignant transformation in 5- 15 per cent of patients over age 40.5 Neurofibromas were present in 52 per cent (n = 32) of all patients. Of this group, 21 patients had neurofibromatosis with GI neurofibromas and no additional neoplasm type identified.

Neurofibroma occurred most frequently as a well-defined nodule and was seen in 71 per cent (n = 15) of the 21 patients with neurofibromas, no other associated neoplasm, and was associated with symptoms of GI bleed or anemia in 52 per cent (n = 11) of patients.

TABLE 2. Location of Gastrointestinal Neoplasms in 61 Reviewed Cases with Neurofibromatosis

TABLE 3. Gastrointestinal Neoplasms in 61 Reviewed Cases with Neurofibromatosis

Although our patient’s neoplasm was benign, malignant neoplasms occurred in 30 per cent (n = 18) of all patients with GI neoplasms and neurofibromatosis. Adenocarcinoma was found most frequently, occurring in 23 per cent of reported cases.

There was a greater than expected incidence of gastrointestinal stomal tumor (GIST) in the reviewed population (6.5%). This is consistent with at least one other study of malignant and benign neoplasms, not limited to GI, in patients with neurofibromatosis where 7 per cent of patients with were found to have GIST.64 GIST has previously been reported in association with neurofibromatosis.54, 67 Recent developments enable neoplasm identification with KIT (CD 117) and treatment of metastatic disease with STI571 (Gleevec), a tyrosine kinase inhibitor. Past diagnosis with light microscope only may have been inadequate to identify correctly these neoplasms, making it possible that some of the neoplasms previously reported as neural or smooth muscle neoplasms were actually GIST.

Treatment of symptomatic GI tumors in patients with NF-1 remains surgical. Due to the high incidence of malignancy and the possibility of malignant degeneration in the more frequently occurring benign neoplasms, all neoplasms should be resected with the use of frozen sections to assure adequate margins. Follow-up in asymptomatic patients consists of annual CBC and hemoccult.36

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61. Costi R, Saruana P, Sarli L, et al. Ampullary adenocarcinoma in neurofibromatosis type 1. Mod Pathol 2001;14:1169-74.

62. Castoldi L, De Rai P, Marini A, et al. Neurofibromatosis-1 and ampullary gangliocytic paraganglioma causing biliary and pancreatic obstruction. Int J Pancreatol 2001;29:93-8.

63. Frank D, Majid N, England D. Massive gastrointestinal bleeding in a patient with von Recklinghausen’s disease: case report. Mil Med 1981;146:438-9.

64. Zoller M, Rembeck B, Oden A, et al. Malignant and benign tumors in patients with neurofibromatosis type 1 in a defined Swedish population. Cancer 1997;79:2125-31.

65. Ruggieri M. The different forms of neurofibromatosis. Childs Nerv Syst 1999;15:295-308.

66. Townsend C. Sabiston Textbook of Surgery: The Biological Basis of Modern Surgical Practice, 16th Ed. Philadelphia: W.B. Saunders, 2001.

67. Min K. Small intestinal stromal tumors with skenoid fibers in neurofibromatosis: report of four cases with ultrastructural study of skeinoid fibers from paraffin blocks. Ultrastruct Pathol 1993;17:307-14.

JENNIFER R. BAKKER, M.D.,* MARIAN M. HABER, M.D.,[dagger] FERNANDO U. GARCIA, M.D.[double dagger]

From the * Department of Surgery, Graduate Hospital, Philadelphia, Pennsylvania; [dagger] Department of Pathology, Drexel University, College of Medicine, Philadelphia, Pennsylvania; [double dagger] Department of Pathology, Drexel University, College of Medicine, Philadelphia, Pennsylvania

Address correspondence and reprint requests to Jennifer R. Bakker, M.D., Graduate Hospital, Pepper Pavillion, Department of Surgery, 18th and Pine, Philadelphia, PA 19146.

Copyright The Southeastern Surgical Congress Feb 2005

Life-Threatening Abdominal Wall Hematoma in a Chronic Renal Failure Patient After a Single Dose of Enoxaparin

We present a patient with chronic renal insufficiency who developed a massive posttraumatic abdominal wall hematoma after a single therapeutic dose of enoxaparin administered during workup of chest pain. Surgical evacuation of the hematoma was required to control life-threatening hemorrhage. Low-molecular-weight heparin use is not without risk and mandates appropriate indication and accurate dosing. Bleeding can occur at any site during heparin therapy, and abdominal wall hematoma should be considered as a source after traumatic injury.

FOR MORE THAN 40 YEARS, heparin has been standard therapy for the initial treatment or prevention of vascular thrombosis. When used appropriately, heparin prevents recurrent thromboembolism with an acceptable risk of major bleeding. Recently, however, the dominance of unfractionated heparin (UH) in the antithrombotic armamentarium has been overtaken by low-molecular-weight heparin (LMWH).

Unfractionated heparin is a complex glycosaminoglycan isolated and purified from porcine intestinal mucosa or bovine lung. It functions as an anticoagulant by inducing a conformational change in the structure of antithrombin (AT), dramatically augmenting its ability to neutralize thrombin, and to a lesser extent, factor Xa and other serine proteases that participate in the formation of fibrin.1 UH binds to endothelial cells and macrophages, as well as to plasma proteins including platelet factor 4 and von Willebrand factor, explaining the variability in plasma heparin activity observed after parenteral administration.

Low-molecular-weight heparin is prepared from UH by enzymatic or chemical hydrolysis, procedures that substantially alter the biological properties and clinical attributes of the anticoagulant. For example, LMWH inhibits factor Xa more effectively than thrombin, lacks nonspecific plasma protein binding, and has a more stable half- life compared to UH.2 LMWH has a more predictable biological availability and is administered in a subcutaneous dose once or twice daily without the need for laboratory monitoring. Because of these characteristics, LMWH is now being used in the emergency department and inpatient and outpatient settings for the treatment or prevention of deep venous thrombosis, pulmonary embolism, and acute coronary syndrome.3

We present the case of a chronic renal failure patient who developed a posttraumatic abdominal wall hematoma after a single dose of enoxaparin.

Case Report

A 43-year-old female (60 kg) with a history of nondialytic chronic renal failure resulting from malignant hypertension presented to the emergency department with chest pain and a blood pressure of 160/90. The differential diagnosis included possible acute myocardial ischemia versus pulmonary embolism. Pertinent and reviewed laboratory results included serum creatinine 4.1 mg/dL, potassium 3.9 meq/L. hematocrit 31 percent, and international normalized ratio (INR) 1.08. After an elevated D-dimer of 2.2 (normal, 0-0.5), the patient received 60 mg of enoxaparin subcutaneously while awaiting ventilation/perfusion (V/Q) scan. The V/Q scan was low probability for pulmonary embolism. Cardiac evaluation including enzymes, and electrocardiogram was also negative, and the patient was discharged. On her way home, the patient was involved in a low-speed motor vehicle crash as a front- seat passenger restrained by shoulder harness and lap belt. She reported increasing lower abdominal pain immediately after the collision and returned to the emergency department.

She arrived hemodynamically stable with examination revealing a large right lower abdominal wall mass in the distribution of the lap belt (Fig. 1). The overlying skin was taught and shining in appearance. CT scan of the abdomen and pelvis revealed a large subcutaneous hematoma of the abdominal wall with no obvious solid organ injury or traumatic hernia (Fig. 2).

FIG. 1. Abdominal wall mass in the distribution of the lap belt.

The patient was emergently transported to the operating room due to the development of hemodynamic instability and rapid expansion of the hematoma. A preoperative hematocrit was 18 per cent, and a low- molecular-weight heparin level (antiXa level) drawn 13 hours after the enoxaparin dose remained elevated at 0.6 IU/mL (therapeutic range, 0.6 to 1.0 antiXa lU/mL). She received 60 mg of protamine sulfate intravenously immediately prior to surgery, and a low- molecular-weight heparin level after the protamine dose was less than 0.1 IU/mL. Evacuation of the hematoma produced approximately 800 mL of blood. Ligation of an actively bleeding superficial inferior epigastgric artery was performed, the hematoma cavity was packed for additional hemostasis, and the patient was transfused 3 units of packed red blood cells. Postoperatively, no further wound bleeding occurred, and the patient was discharged to home on postoperative day 5, performing daily dressing changes. Outpatient follow-up documented complete wound healing by postoperative day 30.

FIG. 2. CT scan revealing subcutaneous abdominal wall hematoma.

Discussion

Rates of serious hemorrhage with UH or LMWH therapy approximate 2 to 6 per cent per patient episode of treatment in clinical trials of acute coronary syndrome and deep venous thrombosis treatment.4, 5 Bleeding occurs more frequently in patients with risk factors including recent surgery or trauma, renal insufficiency, active peptic ulcer disease, hypertension, recent intracranial hemorrhage or stroke, and history of bleeding diathesis.6

If bleeding is severe, protamine sulfate can be used for immediate neutralization of anticoagulant. When the heparin has been given recently, 1 mg of protamine neutralizes approximately 100 IU of UH. The relative prolongations of the activated partial thromboplastin time (PTT) and low-molecular-weight heparin level (antiXa level) are useful for estimating the quantity of circulating heparin and the need for protamine.7

Protamine sulfate is less effective in neutralizing LMWH. One approach is to administer 1 mg of protamine for every 1 mg or 10 antiXa units of LMWH, producing an approximate 50 per cent neutralization of LMWH. Given the longer half-life of LMWH, 4 hours, one-half of this dose can be repeated in 4 hours if necessary.8

Enoxaparin is a LMWH with an average molecular weight of 4-5 kDa compared to 12-15 kDa for UH. It is rapidly absorbed after subcutaneous administration and has a linear dose relationship for antiXa activity over the dose range 20-80 mg. The sustained biological activity of enoxaparin allows less frequent administration than that required for UH, and it is predominantly eliminated by the kidney.9 With a LMWH level within the therapeutic range 13 hours after the emergency department dose, this patient clearly had an impaired clearance and prolonged therapeutic effect of enoxaparin.

After a therapeutic weight-adjusted dose of LMWH is administered by subcutaneous injection, the antiXa activity peaks at approximately 4 hours, and this is the recommended time to perform monitoring assays.2 It should be noted that the measured peak antiXa activity varies among individual LMWH preparations given in the same antiXa dose, due to individual variations in pharmacokinetics. A conservative therapeutic range for peak effect with twice-daily administration of enoxaparin is 0.6 to 1.0 IU/mL for patients being treated for venous thromboembolism.10 In order to avoid an increased risk of bleeding, levels >1.0 IU/mL should be avoided if the appropriateness of the dose is in question in patients with renal impairment.11

The safety of administering standard doses of LMWH to patients with severe renal insufficiency has not been clearly established. Large contemporary randomized controlled trials of LMWH have generally excluded patients with severe renal insufficiency. However, pharmacokinetic and clinical data have become available that allow reasonable conclusions to be made regarding the use of LMWH in these patients. Pharmacokinetic studies have demonstrated that the clearance of the antiXa effect of LMWH is strongly correlated with creatinine clearance (CrCl). This relationship has been shown in single-dose studies of enoxaparin at a CrCl rate of

Evidence-based guidelines from the 7th Annual American College of Chest Physicians Conference on Antithrombotic Therapy recommends using UH to provide full therapeutic anticoagulation therapy in patients with severe renal insufficiency (CrCl

Thromboprophylactic LMWH in patients with renal insufficiency requires separate consideration. Although increased antiXa activity was observed in patients with renal failure who received multiple thromboprophylactic doses of enoxaparin (40 mg daily), the mean peak antiXa level was only 0.6 IU/mL and the trough was

Bleeding can occur at any site during heparin therapy, and abdominal wall hematom\a should be considered as a source after traumatic injury. CT scan of the abdomen can be useful in differentiating subcutaneous from intramuscular abdominal wall hematoma, the latter rarely requiring operative intervention.13 Surgical evacuation of an abdominal wall hematoma may be required to control ongoing hemorrhage or preserve skin viability.

REFERENCES

1. Hirsh J. Warkentin TE, Raschker L. Heparin and low-molecular- weight heparin: mechanisms of action, pharmacokinetics, dosing considerations, monitoring, efficacy, and safety. Chest 1998;114(5 Suppl):489-500.

2. Hirsh J, Raschke R. Heparin and low-molecular-weight heparin: The Seventh ACCP Conference on Antithrombotic and Thrombolytic Therapy. Chest 2004; 126:188-203.

3. Harrison L, McGinnis J. Crowther M. Assessment of outpatient treatment of deep-venous thrombosis with low-molecular-weight heparin. Arch Intern Med 1998;158:2001-10.

4. Gould MK, Dembitzer AD. Sanders GD. Low-molecular-weight heparins compared with unfractionated heparin for treatment of acute deep venous thrombosis, a meta-analysis of randomized controlled trials. Ann Intern Med 1999;130:800-25.

5. Noble S, Spencer CM. Enoxaparin: a review of its clinical potential in the management of coronary artery disease. Drugs 1998;56:259-66.

6. Landefeld CS, McGuire E. Rosenblatt MW. A bleeding risk index for estimating the probability of major bleeding in hospitalized patients starting anticoagulant therapy. Am J Med 1990;89: 569-77.

7. Nieuwenhuis HK, Albada J, Banga JD. Identification of risk factors for bleeding during treatment of acute venous thromboembolism with heparin or low molecular weight heparin. Blood 1991; 78:2337-46.

8. Bergquist D. Born GU, Arfors KE. Efficacy and safety of enoxaparin versus unfractionated heparin for prevention of deep venous thrombosis. Br J Surg 1997;84:1099-103.

9. Noble S, Spencer CM. Enoxaparin: a reappraisal of its pharmacology and clinical applications in the prevention and treatment of thromboembolic disease. Drugs 1995;49:388-400.

10. Boneu B, deMoerloose P. How and when to monitor a patient treated with low molecular weight heparin. Semin Thromb Hemost 2001;27:519-22.

11. Farooq V, Hegarty J, Chandrasekar T. Serious adverse incidents with the usage of low molecular weight heparins in patients with chronic kidney disease. Am J Kidney Dis 2004;43:531- 7.

12. Sanderink GM, Guimart CG, Ozoux ML. Pharmacokinetics and pharmacodynamics of the prophylactic dose of enoxaparin once daily over 4 days in patients with renal impairment. Thromb Res 2002;105:225-31.

13. DuToit DF, Maritz J, Loxton AJ. Reclus sheath hematoma. a complication of anticoagulant therapy. S Afr Med J 1983;63: 902-4.

ROBERT L. WEINSHEIMER, M.D.,* EDWARD LIBBY, M.D.,[dagger] THOMAS R. HOWDIESHELL, M.D.*

From the Departments of * Surgery and [dagger] Medicine, University of New Mexico Health Sciences Center, Albuquerque, New Mexico

Address correspondence and reprint requests to Thomas R. Howdieshell, M.D., F.A.C.S., Trauma/Surgical Critical Care, Department of Surgery, University of New Mexico HSC, Albuquerque, NM 87131.

Copyright The Southeastern Surgical Congress Feb 2005

Cardiovascular Care of Patients With Marfan Syndrome

Summary

Marfan syndrome is a genetic disorder with potentially fatal cardiovascular complications. These risks can be reduced by early diagnosis, appropriate review and timely intervention. Care of patients with Marfan syndrome should be multidisdplinary and multiprofessional. This article reviews the cardiovascular aspects of care and the nursing role.

Key words

* Cardiovascular disorders: prevention and screening

* Cardiovascular system and disorders

These key words are based on the subject headings from the British Nursing Index. This article has been subject to double- blind review.

MARFAN SYNDROME is a genetic, connective tissue disorder. It presents in various body systems: skeletal, ocular, cardiovascular, respiratory, muscular and integumentary (Pyeritz 2000). It was identified in Paris in the late 19th century by Professor Marfan, in a tall, young girl with disproportionately long limbs and arachnodactyly (long digits). These are classic physical signs of the syndrome. Further manifestations were discovered throughout the 20th century (Table 1 ) (De Paepe et al 1996). It is estimated that there are approximately 10,000 patients with Marfan syndrome in the UK, based on an occurrence of one in every 3,000-5,000 live births (van Karnebeek et al 2001). There are no apparent gender or racial differences for the syndrome.

It is caused by mutation in the fibrillin-1 (FBN-1) gene that encodes the glycoprotein fibrillin, on the long arm of chromosome 15. Fibrillin combines with other components to form microfibrils – a fundamental connective structure found throughout the body (Pyeritz 2000). Inheritance is autosomal dominant: there is a 50 per cent chance of an affected parent passing the mutation to his or her child (Pyeritz 2000). It is inherited in approximately 75 per cent of cases and occurs due to spontaneous mutation in the remaining 25 per cent (Pyeritz 2000). Family clusters are common so genetic screening and counselling are required. More than 150 different mutations of the FBN-1 gene have been isolated and it may be that each family has a unique genetic mutation for the syndrome (Pereira et al 1994). This could explain the considerable variability in the clinical presentations of Marfan syndrome. It also means that diagnosis cannot necessarily be made from genetic testing alone.

The wide spectrum of clinical phenotype – the observable characteristics of the genetic condition – and the lack of diagnostic blood tests make definite diagnosis problematic. Expert consensus has resulted in the Ghent diagnostic criteria (Table 1) (De Paepe et al 1996).

There is a subset of Marfan syndrome with severe manifestations which is recognisable at, or soon after, birth. These infants have a high mortality (Morse et al 1990). More typically, patients will be identified as potentially having Marfan syndrome during childhood or following family assessment after the diagnosis of a relative. As connective tissue changes can be confused with normal variation during adolescence, patients may need serial review before a definitive positive or negative diagnosis can be made (Lipscomb et al 1997). Uncertainty over diagnosis can create psychological stress – especially in adolescents. Clinicians need to be aware of this because it will affect concordance with review and treatment programmes. Diagnosis is further complicated by the presence of other related, or similar, conditions where microfibrils are also affected, such as congenital contractural arachnodactyly (CCA); familial mitral valve prolapse (MVP) syndrome; familial aortic dissection; familial ectopia lentis; familial tall stature; and Shprintzen-Goldberg syndrome. The latter is a rare FBN-1 mutation with similar presentation to Marfan syndrome but with more pronounced facial features and additional complications of craniostenosis and ocular proptosis (Pyeritz 2000).

If skeletal changes and body shape have not raised suspicion of Marfan syndrome, then the first clinical presentation is often lens dislocation or joint dislocation. A family history of aortic dissection or sudden unexplained death is important. These events should always raise the possibility of a Marfan syndrome diagnosis and initiate review. As this syndrome affects multiple body systems, review will involve a variety of specialists such as geneticist, cardiologist, ophthalmologist and orthopaedic surgeon (Child and Briggs 2002). Ideally, there should be a clinician experienced in Marfan syndrome coordinating overall care and specialist reviews. In the UK, in practice, services can be disjointed: shared responsibility through different healthcare levels (primary, secondary and tertiary) and clinicians may not always be accompanied by clear coordination. Genetic services and congenital cardiology are usually provided regionally at the tertiary level. There is a need to consider what methods of service delivery are required for this group of patients.

Cardiovascular complications

Patients with Marfan syndrome have serious cardiovascular complications. The most common are aorta dilation, dissection and rupture; aortic valve regurgitation; and MVP and regurgitation (Child and Briggs 2002). There are also reports of tricuspid regurgitation, pulmonary artery dilation, pulmonary valve stenosis, cardiomyopathy and arrhythmias (Adams and Trent 1998). Estimates of the frequency of cardiac complications vary from 50 per cent to 80- 90 per cent of patients with the syndrome (Child and Briggs 2002, Holcomb 2000). The wide variation in estimates reflects the lack of large studies.

Table 1. Ghent diagnostic criteria

Cardiovascular complications represent the most significant threat to life (Dean 2002). In 1972 the median life expectancy for patients with Marfan syndrome was 45 years (Murdoch et al 1972). This had risen to 70 years in a review of 417 patients in 1995. This was attributed to earlier intervention, better diagnosis and the inclusion of patients with milder variants in the study (Silverman et al 1995).

Aorta and aortic valve Aortic aneurysm as a complication of Marfan syndrome was first described by Taussig in 1943 (Gott et al 1999). The extracellular matrix of the arterial wall, mainly in the media layer, is progressively weakened in the syndrome (Child and Briggs 2002). The arterial wall becomes stiffer and less distensible. These changes are systemic to other elastic arteries, resulting in intracranial aneurysms for example, but only dilation in the aorta, and occasionally the pulmonary artery, are clinically significant (Pyeritz 2000).

Dilation is usually located in the ascending aorta, which is under the greatest haemodynamic stress, but can occur in the arch and descending aorta. When dilation is in the area of the sinus of Valsalva there is often aortic valve compromise. Aortic valve regurgitation is rarely seen when the aortic dimension is less than 40mm and is almost always present when the diameter is greater than 60mm (Pyeritz 2000). Aortic dilation is common in patients with Marian syndrome: in a study of 52 children, 43 had aortic dilation (van Karnebeek et al 2001). The same study found 13 of the 52 had aortic valve regurgitation. Aortic dilation is associated with an increased risk of sudden dissection and rupture – emergencies that carry a significant mortality (Silverman et al 1995, Wijetunge 2002). The risk to life is greatest with retrograde dissections because these may compromise the coronary arteries and the pericardium. Patients should be advised of the classic symptoms of aortic dissection – sudden onset severe pain, often located either retrosternally or between the shoulder blades, occasionally in the abdominal midline, ‘tearing’ or ‘sharp’ in nature; pallor; loss of peripheral pulses; paraesthesia; and paralysis – and the need to seek immediate medical assistance.

Aortic dilation is progressive but the natural history of dilation shows a marked variability between patients. It may be that some patients are especially vulnerable genetically or that some additional environmental factors are implicated. While dilation increases the risk of dissection and rupture, they are not inevitable consequences of dilation. Treatment of aortic dilation and aortic valve regurgitation is both medical and surgical. Medical therapy is to reduce haemodynamic strain, usually with beta blockers, and surgical treatment is aortic root replacement and aortic valve replacement. Beta blockers Beta blockers have been used since the 1960s to reduce arterial wall tension in patients with acute aortic aneurysms and dissections. The prophylactic use of beta blockers in patients with Marfan syndrome to slow progressive aortic dilation was first proposed in the 1970s (Halpern et al 1971). Beta blockers might slow progression of aortic regurgitation (Ose and McKusick 1977).

The clinical evidence base for the use of beta blockers in patients with Marfan syndrome is not extensive. There has been one randomised controlled trial of a beta blocker in patients with the syndrome (Shores et al 1994). This compared aortic dilation, as well as clinical endpoints such as aortic dissection and mortality, in 70 patients with Marfan syndrome over a ten-year study: 32 were given propranolol 212mg +/- 68mg; and a control group of 38 had no medicines. The results of the trial showed that high doses of propranolol slowed aortic dilation. There was a \less strong link with the clinical endpoints. During the study ten of the 32 patients taking propranolol experienced side effects – lethargy, depression, insomnia, dream disturbance, heart blocks and mild bronchospasm – although no patient had to stop treatment because of side effects. Further studies have examined the effectiveness of beta blockers in reducing haemodynamic stresses (Reed and Alpert 1992, Yin et al 1989) and in slowing aortic dilation in patients with Marfan syndrome (Rossi-Foulkes et al 1999, Salim et al 1994).

Several questions remain about the prophylactic use of beta blockers for patients with Marfan syndrome:

* Is there a class effect for all beta blockers or are some beta blockers more effective than others?

* Is there an optimum dose?

* Do all patients need prophylactic beta blockers or is there a high-risk sub-group of patients?

* When should beta blockers be started?

* Should they be continued for life?

A complication is that the response of patients with Marfan syndrome to beta blockers is heterogeneous (Haouzi et al 1997, Legget et al 1996, Nollen et al 2004). The incidence of side effects with beta blockers means the benefits of their use need to be considered for individual patients with an appreciation of the risks. One centre recommends using beta blockers if the aortic root is greater than 95 per cent predicted size for that patient’s body size (Salim and Alpert 2001). Another recommendation is that all patients should be considered for beta blockers (Pyeritz 2000). There is also research examining the use of magnetic resonance imaging (MRI) mapping of aortic distensibility as a potential means of identifying patients with Marfan syndrome who have a normal sized but vulnerable aorta and may benefit from early treatment (Groenink et al 2001).

For patients who are unable to take beta blockers, other anti- hypertensives, such as angiotensin-converting enzyme (ACE) inhibitors, have been suggested as an alternative but there are no trials of such drugs in Marfan syndrome (Child and Briggs 2002).

Aorta and aortic valve surgery Surgical treatment of aortic dilation and aortic valve regurgitation is usually by combined aorta graft and prosthetic valve replacement (Gott et al 1999). Benthall and DeBono first described the composite graft valve procedure in 1968 (Treasure 2000). Less commonly, if the sinus of Valsalva is not widely dilated and the valve cusps not stretched, a valve-saving operation is also possible (Pyeritz 2000). While mortality is high in emergency aortic repair, it is low in elective procedures and case studies of patients undergoing this procedure show good long- term outcomes, although absolute numbers remain relatively small. The timing of when to perform surgery has been widely considered in the literature. Expert consensus is that surgery is best undertaken when the indications listed in Box 1 are present (Groenink et al 1999). Late aortic dissections have occurred in patients who have already had aortic repair so there is a need for regular ongoing cardiac review (Treasure and Briggs 2002).

Mitral valve prolapse (MVP) Patients with Marfan syndrome often have MVP, potentially leading to mitral regurgitation, arrhythmias, heart failure and pulmonary hypertension (Pyeritz 2000). In a study of children with Marfan syndrome, MVP occurred in 46 of 52 cases and 25 had mitral regurgitation (van Karnebeek et al 2001). In a study of nine patients with Marfan syndrome and mitral regurgitation all had a dilated annulus, five had a prolapsed mitral valve leaflet and two had ruptured chordae tendineae (Bozbuga et al 2003). Calcification of the mitral valve annulus is also frequently present.

If patients with MVP are asymptomatic and mitral regurgitation is mild or moderate no surgery is indicated. For patients with mitral valve compromise the surgical options are repair or replacement. A review of 160 patients with Marfan syndrome and surgical intervention showed 29 required mitral valve repair and seven mitral valve replacements (Gillinov et al 1994). Repair has been controversial because the underlying connective tissue disorder makes the repair theoretically vulnerable in terms of durability. Repair does have the advantage of preserving the papillary muscles, and therefore left ventricular shape and function, as well as avoiding chronic anti-coagulation. Outcomes are good: in a study of nine patients with Marfan syndrome, mitral regurgitation and left ventricular dilation, surgical procedures on the mitral valve were performed successfully without any complications (Bozbuga et al 2003). However, it should be noted that the cases in the literature are small in absolute numbers so the evidence base is not extensive. For patients with mitral valve disorders and heart failure, medical treatment would be as recommended for all cases of heart failure.

Other cardiac complications It has been suggested that not all cases of sudden death in patients with Marfan syndrome may be due to aortic dissection. Arrhythmias and cardiomyopathy have both been considered as additional causative factors.

Patients with Marfan syndrome may have supra-ventricular and ventricular dysrhythmia and the incidence rises with MVP (Pyeritz 2000). Arrhythmias were considered in a longitudinal study of 70 patients with Marfan syndrome who were followed up for 24 years (Yetman et al 2003). All had cardiovascular involvement identified at diagnosis and all were started on beta blockers. Although none died of aortic dissection, 4 per cent (n=3) died of an arrhythmia. The authors linked these deaths to the patients having enlarged left ventricles, MVP and repolarisation abnormalities (Yetman et al 2003).

It seems that some patients with Marfan syndrome may have an unusual pattern of trans-mitral valve diastolic blood flow with reduced ventricular compliance and myocardial contraction. Diastolic dysfunction, in a study of 28 patients with Marfan syndrome, differed from 28 controls (patients without Marfan syndrome) and was independent of valve disease (Porciani et al 2002). Cardiomyopathy, without valve involvement, may be due to defects in the cardiac muscle (Pyeritz 2000).

Cardiovascular assessement and review

Patients being assessed for Marfan syndrome should all have an initial cardiovascular consultation with a clinician experienced in the syndrome. Clinical assessment is aimed at assisting the diagnosis by establishing major and minor cardiovascular criteria of the Ghent diagnostic criteria (De Paepe et al 1996). Investigations will establish baselines of cardiac structure and function to guide follow-up reviews.

History taking should include a thorough family history. It is important to differentiate hereditary from spontaneous mutations and to map the family tree so that assessment of other family members can take place. A history of a family member with aortic dissection or an unexplained sudden death is both a potential major diagnostic criteria and a risk factor for future complications. It must be remembered that most isolated family histories of aortic aneurysms, scoliosis, tall stature and myopia are not caused by Marfan syndrome (Pyeritz 2000). Symptoms and functional capacity need to be established: dyspnoea and fatigue could suggest heart failure from mitral regurgitation, for example.

A careful cardiovascular examination should be performed. Cardiac apex beat should be palpated for downward and left lateral displacement that may suggest left ventricular hypertrophy or a sternal heave that may suggest right ventricular hypertrophy. Cardiac auscultation may reveal MVP as a midsystolic click and mitral regurgitation as a pansystolic murmur heard best at the apex. Aortic regurgitation may be heard best as an early diastolic murmur nearer the base. Changes in the aorta can be suggested if there are differences in blood pressure between the upper limbs or radio- femoral pulse delays, although absence of these signs does not rule out aortic dilation or coarctation. Blood pressure needs accurate measurement because hypertension will initiate active treatment.

Box 1. Indications for aortic surgery

Investigations should include a minimum of 12-lead electrocardiogram (ECG) and transthoradc echocardiogram. On ECG evidence of arrhythmias, hypertension or hypertrophy may be present – although there are no ECG patterns or changes specific to Marfan syndrome (Pyeritz 2000). The presence of skeletal abnormalities – such as pectus excavatum – might affect the cardiac axis and the voltage recorded by the electrodes. A transthoracic echocardiogram should be performed to assess chamber sizes, valve structure and function, flow across valves and the aortic size at several points, including measurement across the sinus of Valsalva. Echocardiogram measurements need to be applied against a nomogram of body surface area to take into account different heights and weights, especially in children – these are available online (www.marfan.org). As there are technological limits to the accuracy of transthoracic echocardiogram and skeletal abnormalities can make transthoracic echocardiogram difficult, there may be a role for other scanning modalities – tranoesphageal echocardiogram, computerised tomography and MRI – and scanning further down the aorta in some cases (Leech 2002).

For patients with a clear diagnosis of Marfan syndrome but no cardiovascular compromise, an annual cardiovascular review of echocardiogram and clinical assessment is recommended (Child and Briggs 2002, Dean 2002). If patients have cardiovascular compromise – in terms of symptoms, valve dysfunction or an aorta greater than 40mm – it is recommended that review is not less than every six months (Child and Briggs 2002). Women who are pregnant and have Marfan syndrome should be frequently reviewed jointly with an obstetrician.

Nursing role

On diagnosis and on review, patients and their families need information about th\eir condition and assistance with the psychosocial adaptations needed to cope with the disease. Specific advice should be given about sports, dental procedures, family and work-related issues and what to do in an emergency. Acute aortic dissection is rare but patients and their families should be advised that sudden thoracic pain, especially a tearing pain in the back, associated with dizziness or loss of consciousness, requires urgent transfer to hospital by ambulance. The appropriateness of beta blockers in individual cases should be discussed and the indications for surgical referral explained. These are all areas where nursing skills can be valuably employed. Patients are likely to be reviewed most frequently by the cardiac team, and much specific advice is given to avoid cardiac complications, so it would seem logical for the cardiac team to act as key clinicians alongside the primary care team. Current UK service provision does not include many cardiac specialist nurses in adult congenital and genetic heart disease clinics. Little has been published by nurses on the syndrome in the UK and there is a need for research to consider what nursing interventions would be most effective in this patient group. Nursing staff need to raise their awareness of Marfan syndrome to help identify patients and reduce under-diagnosis, to provide psychological support to patients and their families and to be vigilant for complications when they nurse patients with Marfan syndrome.

Psychosocial issues People with Marfan syndrome often have distinctive physiognomy and may be sensitive about their appearance. Many report being bullied and teased at school (van Tongerloo and De Paepe 1998). They have to adapt psychologically to having a chronic and progressive disorder, which may or may not make them feel unwell but is potentially fatal. In a study of 17 patients aged under 35, the condition had an impact on physical activities, schooling and job opportunities, although most patients felt they were coping well (van Tongerloo and De Paepe 1998). This was confirmed by a large study of 174 patients with Marfan syndrome in the United States who reported psychological issues but an overall adequate quality of life (Peters et al 2002).

The disorder has implications for patients’ reproductive choices and relationships and these should be sensitively discussed alongside genetic counselling (Ryan-Krause 2002). Feelings of guilt about passing on the condition to children, or of being a family member who has been found not to have the condition, have been reported (Ryan-Krause 2002). There is some research that has suggested a link between attention deficit hyperactivity disorder (ADHD) and Marfan syndrome (Hofman et al 1988, Lannoo et al 1996).

Sports advice One of the common referral scenarios is of young athletes who have had medical examinations that raised the possibility of Marfan syndrome due to skeletal signs (Ryan-Krause 2002). The tall stature and long limbs often present in the disorder are especially useful for sports such as basketball, volleyball and some athletics. A special gift for these sports also means special risks: ocular, skeletal and cardiovascular. An elongated globe stretches the retina and cilia muscles and makes lens dislocation likely. Ligament laxity makes joint dislocations likely. Static high- demand sports, like weight lifting, place extra strain on the aortic wall and this increases the risk of dissection. Up to 10 per cent of aortic dissections in patients with Marfan syndrome follow physical exertion (Child and Briggs 2002). Patients who have had valve surgery are at increased risk of bleeding and bruising from contact sports due to the use of anti-coagulants. Patients with Marfan syndrome should be advised to avoid contact sports, static high- demand sports and wear eye protection for racket sports (Salim and Alpert 2001).

Dental advice Patients with valvular heart disease are at increased risk of endocarditis. They should take prophylactic antibiotics peri-operatively and to cover invasive dental work (Bonow et al 1998). Because patients with Marfan syndrome may have valve abnormalities and are also more likely than average to require dental work – if they have high arched palates and teeth crowding – they should be advised on the need for prophylactic antibiotics before dental work likely to result in bleeding (Ryan-Krause 2002).

Pregnancy advice and management Patients with Marfan syndrome face two important issues related to having children. First, because the condition has autosomal dominant inheritance, there is a 50 per cent chance of the gene being transferred from parent to child (Elkayam et al 1995). It is also possible that the child may have a more severe or complex presentation than the affected parent. The second issue is that pregnancy can increase the risk of aortic dissection in women with Marfan syndrome (Rahman et al 2003). Four aortic dissections were reported in a study of 91 pregnancies in women with Marfan syndrome (Lipscomb et al 1997) and another series of 24 pregnancies reported two aortic dissections (Rahman et al 2003). All women have a risk of aortic dissection in the third trimester and a month post-partum but the risks appear higher in women with Marfan syndrome.

A review of the literature found more than 30 cases of cardiovascular complications during pregnancy (Elkayam et al 1995). It is thought this risk is highest in women with pre-existing cardiovascular complications before pregnancy. A study of 45 pregnancies in 21 women with Marfan syndrome found two aortic dissections – which occurred in women already identified as having cardiovascular compromise before pregnancy – and found no evidence of increased risk of longer-term problems (Rossiter et al 1995). The increased dissection risk may be due to hyperdynamic strain, hormonal changes and structural changes in the arterial wall during pregnancy.

While pregnancy is usually complication-free for women with Marfan syndrome, it cannot be guaranteed and it is recommended that special measures are taken: pre-pregnancy counselling about genetic inheritance and pregnancy risks to mother and fetus; scanning of the aorta to include thoracic and abdominal aorta; repeat echocardiograms each trimester and review jointly by cardiologist and obstetrician. Any evidence of aortic dilation means Caesarean section – if the fetus is viable – or termination of pregnancy. Cardiac surgery can be performed as required after delivery or termination. Various beta blockers have been used in pregnancy (propranolol, atenolol and metoprolol) but there is no clear recommendation for routine use (Pyeritz 2000).

Work-related issues

Patients are advised to avoid work that involves repetitive heavy lifting to minimise excessive aortic wall strain. As fatigue is a commonly reported symptom in Marfan syndrome this may affect career decisions. In the UK fitness to drive is regulated by the Driver and Vehicle Licensing Agency (DVLA). Patients with Marfan syndrome need to notify the DVLA if they have a Group 2 licence (public service vehicles and heavy goods vehicles) and must be able to demonstrate annual cardiac reviews – the presence of an aortic root greater than 55mm will disbar the driver until satisfactory treatment. In other circumstances, such as being symptomatic, patients may need medical advice on their suitability to continue driving and the rules are available at the DVLA website (www.dvla.gov.uk). Patients should also be advised on the necessity of informing insurers of their diagnosis as failure to disclose may invalidate policies.

Conclusion

Marfan syndrome is a complex disorder with potentially fatal complications. The most serious complications are cardiovascular. The improvement in prognosis over the past 30 years suggests regular cardiac review, advice and timely intervention are important and beneficial (Silverman et al 1995). The nature of the cardiovascular review means that there are roles naturally suited to the skills of doctors, nurses and cardiac physiologists. As the cardiovascular complications are the most serious and need annual review, it is likely the cardiac team will be the most active clinicians once the diagnosis has been made. The involvement of other specialties such as genetics, orthopaedics, ophthalmology and psychology is likely to be more episodic, depending on particular needs. Links with patient groups and associations can provide a means of involving patients in the service in a more integrated manner.

There are a limited number of doctors with an interest in Marfan syndrome and patients with genetic heart disease have traditionally been cared for alongside patients with congenital heart disease in tertiary cardiac centres. This may not be the only model of care in the future. It has the advantage of specialism and availability of scanning and other resources but has the disadvantage of often significant journeys for the patient and oversubscribed clinics.

Awareness of Marfan syndrome is important for all healthcare staff because it is thought to be under-recognised and diagnosed. To assist in planning services and to consider the effectiveness of interventions further research is necessary to consider service needs from a patient perspective and to consider how the nursing role can be best deployed

Nicholson C (2005) Cardiovascular care of patients with Marfan syndrome. Nursing Standard. 19, 27, 38-44. Date of acceptance: December 8 2004.

Online archive

For related articles and author guidelines visit our online archive at:

www.nursing-standard.co.uk and search using the key words above.

Further reading and resources

The Marfan Syndrome: A Clinical Guide (Child and Briggs 2002) is short and informative and is available from the British Heart Foundation website (www.bhf.org.uk)

Driver and Vehicle Licensing Agency

www.dvla.gov.uk

Grown Up Congenital Heart Patients Assoc\iation

www.guch.org.uk

Marfan Association UK

www.marfan.org.uk

National Marfan Foundation

www.marfan.org/nmf/index.jsp

REFERENCES

Adams J, Trent R (1998) Aortic complications of Marfan’s syndrome. Lancet. 352, 9142, 1722-1723.

Bonow R et al (1998) Management of patients with valvular heart disease. AHA/ACC practice guidelines. Journal of American College of Cardiology. 32, 5, 1486-1588.

Bozbuga N et al (2003) Surgical management of mitral regurgitation in patients with Marfan syndrome. Journal of Heart Valve Disease. 12, 6, 717-721.

Child A, Briggs M (2002) The Marfan Syndrome: A Clinical Guide. London, British Heart Foundation.

De Paepe A et al (1996) Revised diagnostic criteria for the Marfan syndrome. American Journal of Medical Genetics. 62, 4, 417- 426.

Dean J (2002) Management of Marfan syndrome. Heart. 88, 1, 97- 103.

Elkayam U et al (1995) Cardiovascular problems in pregnant women with the Marfan syndrome. Annals of Internal Medicine. 123, 2, 117- 122.

Gillinov A et al (1994) Mitral valve operation in patients with the Marfan syndrome. Journal of Thoracic and Cardiovascular Surgery. 107, 3, 724-731.

Gott V et al (1999) Replacement of the aortic root in patients with Marfan’s syndrome. New England Journal of Medicine. 340, 17, 1307-1313.

Groenink M et al (2001) Biophysical properties of the normal- sized aorta in patients with Marfan syndrome: evaluation with MR flow mapping. Radiology. 219, 2, 535-540.

Groenink M et al (1999) Survival and complication-free survival in Marfan’s syndrome: implications of current guidelines. Heart. 82, 4, 499-504.

Halpern B et al (1971) A prospectus on the prevention of aortic rupture in the Marfan syndrome with data on survivorship without treatment. Johns Hopkins Medical Journal. 129, 3, 123-129.

Haouzi A et al (1997) Heterogeneous aortic response to acute beta- adrenergic blockade in Marfan syndrome. American Heart Journal. 133, 1, 60-63.

Hofman K et al (1988) Marfan syndrome: neuropsychological aspects. American Journal of Medical Genetics. 31, 2, 331-338.

Holcomb S (2000) Marfan syndrome: a review. Dimensions of Critical Care Nursing. 19, 4, 22-23.

Lannoo E et al (1996) Neuropsychological aspects of Marfan syndrome. Clinical Genetics. 49, 2, 65-69.

Leech G (2002) Echocardiography in diagnosis and management. In Child A, Briggs M (Eds) The Marfan Syndrome: A Clinical Guide. London, British Heart Foundation.

Legget M et al (1996) Aortic root complications in Marfan’s syndrome: identification of a lower risk group. Heart. 75, 4, 389- 395.

Lipscomb K et al (1997) Evolving phenotype of Marfan’s syndrome. Archives of Disease in Childhood. 76, 1, 41-46.

Morse R et al (1990) Diagnosis and management of infantile Marfan syndrome. Pediatrics. 86, 6, 888-895.

Murdoch J et al (1972) Life expectancy and causes of death in the Marfan syndrome. New England Journal of Medicine. 286, 15, 804-808.

Nollen G et al (2004) Aortic pressure-area relation in Marfan patients with and without beta blocking agents: a new non-invasive approach. Heart. 90, 3, 314-318.

Ose L, McKusick V (1977) Prophylactic use of propranolol in the Marfan syndrome to prevent aortic dissection. Birth Defects Original Article Series. 13, 3C, 163-169.

Pereira L et al (1994) A molecular approach to the stratification of cardiovascular risk in families with Marfan’s syndrome. New England Journal of Medicine. 331, 3, 148-153.

Peters K et al (2002) Living with Marfan syndrome III: quality of life and reproductive planning. Clinical Genetics. 62, 2, 110-120.

Porciani M et al (2002) Diastolic subclinical primary alterations in Marfan syndrome and Marfan-related disorders. Clinical Cardiology. 25, 9, 416-420.

Pyeritz R (2000) The Marfan syndrome. Annual Review of Medicine. 51, 481-510.

Rahman J et al (2003) Obstetric and gynecologic complications in women with Marfan syndrome. The Journal of Reproductive Medicine. 48, 9, 723-728.

Reed C, Alpert B (1992) Assessment of ventricular performance after chronic beta-adrenergic blockade in the Marfan syndrome. American Journal of Cardiology. 70, 4, 541-542.

Rossi-Foulkes R et al (1999) Phenotypic features and impact of beta blocker or calcium antagonist therapy on aortic lumen size in the Marfan syndrome. American Journal of Cardiology. 83, 9, 1364- 1368.

Rossiter J et al (1995) A prospective longitudinal evaluation of pregnancy in the Marfan syndrome. American Journal of Obstetrics and Gynecology. 173, 5, 1599-1606.

Ryan-Krause P (2002) Identify and manage Marfan syndrome in children. Nurse Practitioner. 27, 10, 26-37.

Salim M, Alpert B (2001) Sports and Marfan syndrome: awareness and early diagnosis can prevent sudden death. The Physician and Sportsmedidne. 29, 5, 80-85.

Salim M et al (1994) Effect of beta-adrenergic blockade on aortic root rate of dilation in the Marfan syndrome. American Journal of Cardiology. 74, 6, 629-633.

Shores J et al (1994) Progression of aortic dilation and the benefit of long-term beta-adrenergic blockade in Marfan’s syndrome. New England Journal of Medicine. 330, 19, 1335-1341.

Silverman D et al (1995) Life expectancy in the Marfan syndrome. American Journal of Cardiology. 75, 2, 157-160.

Treasure T (2000) Cardiovascular surgery for Marfan syndrome. Heart. 84, 6, 674-678.

Treasure T, Briggs M (2002) Thoracic aorta surgery in Marfan syndrome. In Child A, Briggs M (Eds) The Marfan Syndrome: A Clinical Guide. London, British Heart Foundation.

van Karnebeek C et al (2001 ) Natural history of cardiovascular manifestations in Marfan syndrome. Archives of Disease in Childhood. 84, 2, 129-137.

van Tongerloo A, De Paepe A (1998) Psychosocial adaptation in adolescents and young adults with Marfan syndrome: an exploratory study. Journal of Medical Genetics. 35, 5, 405-409.

Wijetunge D (2002) Diagnosis and management of acute complications associated with Marfan syndrome: pitfalls in the emergency setting. In Child A, Briggs M (Eds) The Marfan Syndrome: A Clinical Guide. London, British Heart Foundation.

Yetman A et al (2003) Long-term outcome in patients with Marfan syndrome: is aortic dissection the only cause of sudden death? Journal of the American College of Cardiology. 41, 2, 329-332.

Yin F et al (1989) Arterial hemodynamic indexes in Marfan’s syndrome. Circulation. 79, 4, 854-862.

Christopher Nicholson RGN, DipHE, BA(Hons), MSc, is cardiac nurse specialist, heart failure, Preston Primary Care Trust, Preston.

Email: [email protected]

Copyright RCN Publishing Company Ltd. Mar 16-Mar 22, 2005

Fatal Family Trait – Hypertrophic Cardiomyopathy, a Hereditary Heart Disease, Strikes Canton Siblings

Scalf, who for three years has had a pacemaker/defibrillator implanted in her chest, worries less now about her own sudden death than of her younger brother, her own daughter and even her nieces.

“Am I gonna die?” Joshua Lane wondered loud enough for everyone in the local Canton cafe to hear. “I’m 24. I shouldn’t be thinking about stuff like this. I shouldn’t have to be dealing with stuff like this. …

“I was looking forward to getting older and learning new things about myself. But looking at us now, it doesn’t look like that’s going to happen. My brother didn’t ever reach his 30th birthday.”

Added Scalf bluntly: “He was 29 years, 29 days.”

Hypertrophic cardiomyopathy, or HCM, is diagnosed through the use of an echocardiogram that can reveal the thickening of the walls of the heart that ultimately causes the heart to not function properly. Genetic testing can diagnose the hereditary disease, but not the extent of the symptoms or how severe the 200-some mutations will appear in each individual, according to Robert Bonow, chief of cardiology at Northwestern Memorial Hospital and a professor of medicine at Northwestern University.

Bonow, the former president of the American Heart Association, said the real problem is the disease is tricky. And genetic testing to confirm diagnosis is expensive.

“It can be misdiagnosed,” he said. “It can masquerade like a lot of other more common disorders.”

Scalf and Zack Lane both had formal diagnoses. Their maternal grandfather also is believed to have died from the disease at 34. Their father has numerous heart conditions, but no formal diagnosis. And Joshua Lane and Scalf’s daughter both show numerous warning signs.

HCM can look like a heart attack. It can appear as asthma. It can appear as a typical heart murmur. It even is often diagnosed as anxiety disorder or panic attacks, according to the Hypertrophic Cardiomyopathy Association, or HCMA, founded by Lisa Salberg of New Jersey.

Salberg, too, has a life-saving pacemaker/defibrillator. Four of her family members have died from HCM, and four other members currently live with it.

“Unfortunately,” Bonow said in most cases, “it’s not diagnosed at all until something catastrophic happens.”

Bonow said the disease affects less than 1 percent of the population; however, busy cardiologists see it frequently. “Lots of the stories you hear of young athletes dying on the playing field are dying from this condition.”

And most of the time they were misdiagnosed, he said.

Still, the disease has been studied for more than 50 years. Bonow recommends if any family has had a member die unexpectedly from a heart condition under the age of 55, the entire family needs to be screened for HCM. If there is any family history, “I would recommend the family get screened.”

Risk factors

Bonow said most cardiologists require two of the major risk factors for sudden death before implanting a pacemaker device. Those factors include: family history, cardiac arrhythmia, massive thickness (septum measuring more than 3 mm), adverse blood pressure response or prior cardiac arrest. Some doctors are very comfortable with diagnosing someone with only one of these risk factors, he said.

This is where Scalf said her family is frustrated. Her 8-year- old daughter Samantha has all the warning signs, numerous symptoms and the all-important family history. Yet, Scalf feels her cardiologists aren’t taking the situation seriously enough.

“She’ll be sitting at the table, she’ll grab her chest and her heart will just start racing,” Scalf said of her youngster. “It’s just the same as me.”

Scalf also has a 5-year-old son. That age is typically too young to exhibit major symptoms, but Scalf wants aggressive testing as he gets older.

Even with years of study, Bonow admits there are areas throughout the country with less awareness of the disease. People who suspect they have the disease need to see a specialist.

While genetic testing can reveal up to 10 different genes that can mutate to cause the disease, Salberg said it’s not until puberty when the thickening of the heart walls begins to show up and can be revealed on an echocardiogram.

Joshua Lane suffers from frequent racing heart rate, shortness of breath, chest pain and more. He was diagnosed with athletically induced asthma while in high school. Without the HCM diagnosis, however, Joshua cannot get the pacemaker/defibrillator that has shocked his sister’s heart back into rhythm 18 times.

According to Scalf, doctors told the family Zack’s HCM wasn’t serious enough to warrant a pacemaker.

“They told my mother that the form Zack had was non-life threatening,” Scalf said, adding now they’ve cremated his remains and worry about his children. The family could hardly find a picture of the former Maytag worker, turned college student, without one or both of his girls in his arms.

“I believe if he had a pacemaker, he’d still be alive today,” Joshua Lane said.

The death of her sister and the same lack of awareness of the disease brought Salberg to form HCMA in 1996.

The problem is the disease is so variable, she explains, agreeing that an affected family can have one member with the genes but no symptoms and another family member completely debilitated with the disease.

“We don’t know why it’s so debilitating in some and in others so non-chalant,” Salberg said.

Basically, with HCM the cell structure in the heart is improper, Salberg said. There are 10 different genes with over 200 different mutations that cause the same disease. And just because someone has the gene doesn’t mean they will exhibit the disease. Carriers have a 50-50 chance of passing the disease on to their children.

“There is a misunderstanding in the medical community,” Salberg said of the disease, which is more common than cystic fibrosis or muscular dystrophy. “We don’t understand all the risk factors well enough to determine the risk of sudden death.”

“… The disease needs to be taken more seriously. We understand the disease more than we did 10 years ago. We know there are a lot of people out there at risk and don’t know it. And we don’t want to lose any more young people. I would think (Zack) would have had a defibrillator.”

For more information on HCM, go to www.4hcm.org.

Use of Domperidone As a Prokinetic and Antiemetic

Domperidone is a synthetic benzimidazole compound that is used as a prokinetic agent for treatment of upper gastrointestinal (GI) motility disorders and as an antiemetic. This compound also is gaining popularity as a galactagogue. Domperidone is a specific dopaminei receptor (D2) antagonist that does not cross the blood- brain barrier. It exerts its effect at peripheral D2 receptors in the GI tract; the chemoreceptor trigger zone (CRT), which is outside the blood-brain barrier; and the pituitary.1-4 The basic pharmacology of domperidone and its use as a galactagogue have been reviewed in this journal and elsewhere.5 7 This article provides background information for use of domperidone as an antiemetic, with a focus on Parkinson’s disease, and as a prokinetic agent, with a focus on diabetic gastropathy.

Domperidone as An Antiemetic

Background

Vomiting is an organized process that is coordinated by an area of the medulla called the vomiting center or central pattern generator. The vomiting center receives input from several sources, including the CRT, GI tract (stomach and small intestine), higher cortical centers of the brain and the labyrinths, and orchestrates the gastromotor functions that result in vomiting. Domperidone’s action as an antiemetic is thought to stem from antagonism of D2 receotors at the CRT.1,3,8,9

Parkinson’s Disease

Parkinson’s disease is a progressive movement disorder characterized by degeneration of dopaminergic neurones in the substantia nigra of the midbrain. Treatment is aimed at increasing dopaminergic stimulation of the striatal neurons involved in controlling movement, using dopamine-increasing agents or dopamine agonists.'” Concomitant stimulation of dopamine receptors at the CRT and, possibly, in the GI tract results in the common side effects of nausea and vomiting. Domperidone is used prophylactically to counteract these effects.11-12

Levodopa (L-Dopa), a precursor of dopamine, is a mainstay in the treatment of Parkinson’s disease. Oral domperidone (10 to 20 mg 3 times a day) has been shown to reduce levodopa-induced nausea and vomiting.13

Domperidone (60 mg daily) reduced the incidence of nausea and vomiting in a placebo-controlled study of patients receiving bromocriptine (a dopamine receptor agonist) therapy and allowed higher doses of bromocriptine to be tolerated.14

Parkinson’s disease is characterized by unpredictable “off periods of poor drug response and increased parkinsonian symptomatology. Management of off periods with apomorphine (intermittent injections or continuous infusion), a short-acting dopamine receptor agonist, was made practical by the use of domperidone to counteract apomorphine’s potent emetic effects. Domperidone pretreatment (10 to 20 mg by mouth 3 times a day) is typically started 1 to 3 days before apomorphine treatment is begun. Patients can often gradually reduce or stop domperidone after a few weeks, as tolerance develops to apomorphine’s emetic effects, possibly due to down regulation of dopamine receptor sensitivity in the medulla (vomiting center) after continuous dopaminergic stimulation.IM”

Domperidone (20 mg 3 times a day) has been used to allow quicker titration of pergolide as an adjunct to L-Dopa therapy.20 Hobson et al:l suggest domperidone as an adjunct to ropinirol or pramipexole therapy, two newer dopamine agonists that are relatively specific for D2 receptors.

Surgery-Postsurgical, Chemotherapy- or Radiation-Induced Emesis

Early studies of domperidone’s utility for postsurgical, and chemotherapy- or radiation-induced emesis used intravenous (IV) or intramuscular administration (reviewed elsewhere).4However, parenteral forms of domperidone were withdrawn worldwide following reports of severe adverse cardiac effects.l: Oral domperidone has seen limited use in treatment and prevention of postoperative nausea.22 In general, serotonin receptor antagonists are now recommended as first-line treatment of chemotherapy- or radiation- induced emesis; dopamine receptor antagonists may have a place as alternative or adjunct therapy.8,23-25

Prokinetic Activity of Domperidone

The stomach’s function of receiving, preparing and delivering nutrients to the small intestine in a usable form is a complex process in which the central, autonomie and enteric nervous systems all play a role. Briefly, the main anatomical parts of the stomach are the cardia, fundus, body and antrum. At the beginning of a meal, the fundus relaxes to make room tor the incoming food. After a lag phase, regular contractions of the antrum mix food particles with acid and pepsin into a suspension called chyme, which is then emptied through the pyloric sphincter into the duodenum, the first part of the small intestine. The gastric emptying rate depends upon the physical and chemical characteristics of the stomach’s contents; typical gastric emptying time is 1 to 4 hours. In the interdigestive period, four phases of spontaneous gastric contractions, some of which are controlled by pacemaker cells of the stomach (interstitial cells of Cajal), clear the stomach of undigestible solids.26

Dopamine is synthesized in the GI tract, spleen and pancreas in humans; Dl-like and D2-like receptors have been demonstrated in several mammals at various GI sites. Dopamine’s effect on the GI tract is primarily inhibitory, as evidenced by reduced lower esophageal sphincter tone, reduced gastric tone and intragastric pressure, and decreased antroduodenal coordination.1,3,26-28

Dopamine appears to directly inhibit gastric muscle contractions via postjunctional muscular D2 receptors in the lower esophageal sphincter, fundus and antrum.1,3,27 Domperidone’s prokinetic effect is likely a result of blockade of D2 inhibitory receptors. It has been shown to increase lower esophageal sphincter pressure, improve antroduodenal coordination and gastric emptying and normalize gastric dysrhythmias.28-32

Dopamine also has an indirect inhibitory effect via inhibition of cholinergic transmission in the myenteric plexus, which regulates the circular and longitudinal layers of smooth muscle. This inhibition was shown in isolated guinea pig stomach to be sensitive to domperidone, indicating mediation by prejunctional D2 receptors on postganglionic cholinergic neurons.” w However, domperidone has not been shown to have procholinergic activity in isolated human stomach.1-3,35

Diabetic Gastropathy

Diabetic gastropathy is an umbrella term for a constellation of upper GI symptoms in patients with diabetes, including nausea, vomiting, bloating, abdominal discomfort and early satiety or fullness, suggestive of underlying neuromuscular dysfunctions of the GI tract. Potential neuromuscular dysfunctions include gastroparesis (delayed gastric emptying), gastric dysrhythmias, antralhypomotility and dilation, reduced antroduodenal coordination and gastric tone dysfunction26,36-37

The correlation between upper GI symptoms and the rate of gastric emptying is relatively weak, thus gastroparesis is considered to be an indicator of gastroduodenal motor abnormality, rather than the sole cause of the symptoms.3,26,29,36 Upper GI symptoms may affect up to 50% of patients with diabetes, but symptoms do not necessarily predict delayed gastric emptying.3,26,37 Conversely, patients with diabetes who have poor gastric emptying (up to 50% of patients with type 1 diabetes) may have no upper GI symptoms, although they may experience poor glycemie control due to mismatch of insulin administration with emptying of nutrients into the small bowel.3,26,36,37

The etiology of diabetic gastropathy is not well understood. Autonomie neuropathy and hyperglycemia are thought to be two dominant factors.

Autonomie Neuropathy

Yagal nerve impairment might alter antroduodenal inotility, gastroesophageal reflux activity and gastric secretory function. Although the association between gastroparesis and abnormal autonomie function is relatively weak, it is thought to be a contributing factor.26,36-37

Hyperglycemia/Glucose Toxicity

An inverse relationship has been shown between gastric emptying and blood-glucose concentration both in healthy individuals and in those with type 1 or type 2 diabetes. In addition, acute changes in blood-glucose concentration can alter the perception of sensations from the stomach and duodenum.26,36-37

Other Factors

Knteric neuropathy, altered postprandial release of hormones and neurotransmitters, visceral hypersensitivity and psychological disorders may potentially contribute to gastric dysfunction in patients with diabetes. In addition, there are many other causes of gastroparesis besides diabetes.26,36-37

Evaluation of Patients

Evaluation of patients with diabetes who present with upper GI symptoms should include a complete physical examination and medical history. Other causes of upper GI symptoms and poor gastric emptying, such as mechanical obstruction, poor glucose control or medications that slow gastric emptying, eg, anticholinergics and dopaminergics, should be ruled out.26,36-37 Gastric scintigraphy (direct measure of the transit time of a radiolabeled meal) is the most common, and considered to be the most accurate, way to evaluate gastric emptying.36 Other methods for evaluating gastric myoelectrical events include electrogastrophy, scintigraphic breath tests, ultrasonography, magnetic resonance imaging and antroduodenal manometry.37

Tre\atment

Treatment focuses on improving upper GI symptoms, improving glycemie control and optimizing nutritional intake and quality of life. Optimizing glucose control and avoiding drugs that can slow gastric emptying, followed by dietary and lifestyle modifications, are the usual initial course of treatment. Common recommendations include smaller, more frequent meals that are low in fat and fiber (to avoid bezoar formation); and increased nutrient liquids, although controlled trials have not proven this approach to be effective. Moderate exercise is encouraged, as this accelerates gastric emptying.-26,36-37

Prokinetic agents are the mainstay of pharmacological treatment. Mechanisms of action include dopamine D2 receptor blockade (domperidone and metoclopramide), stimulation of 5HT4 receptors (metoclopramide and cisapride) and stimulation of motilin receptors (erythromycin).36 Placement of a feeding jejunostomy tube is a treatment of last resort.26,36-37

Efficacy

Numerous clinical trials have consistently shown oral domperidone, typically 20 mg 4 times a day, to improve symptom scores, improve gastric emptying, correct gastric dysrhythmias, decrease hospitalizations and improve the quality of life in patients with diabetic gastropathy or gastroparesis.2-3,26,29-30,38- 44 The complex etiology of diabetic gastropathy, and the weak association between more objective measurements of gastroduodenal function (gastric emptying, electrogastrographic measurements) and symptoms, complicate interpretation of these studies. Domperidone’s antiemetic function must also be considered in assessing symptom improvement.

Symptomatic improvement is the most common outcome evaluated in clinical trials of domperidone efficacy. Other parameters include measurement of gastric emptying, gastric myoelectrical rhythm recording, health-related quality of life assessment and hospitalization rates. Many studies have methodological limitations related to one or more parameters: small study size, lack of reporting of patients’ blood glucose levels, lack of placebo control, lack of randomization, unblinded treatment or nonvalidated measurement of symptoms.29,36,38-39 A meta-analysis39 of clinical studies of four prokinetics (cisapride, metoclopramide, domperidone and erythromycin) showed that open-trial and single-blind studies showed greater improvement in both symptom assessment and gastric emptying times than did double-blind trials, indicating a substantial placebo effect and/or bias. The use of domperidone and metoclopramide in the treatment of gastroparesis is currently under review by the Cochrane Upper Gastrointestinal and Pancreatic Diseases Group.4′

The largest clinical trial4042 used a two-phase withdrawal design to enrich the trial with treatment responders. In phase I, 269 insulin-dependent patients with diabetes who had symptoms of gastroparesis [≥8 out of 15 maximum in total symptom score (TSS)] received single-blind treatment with domperidone 20 mg 4 times a day for 4 weeks. Patients were classified as responders (n = 208, 77%) if they showed statistically significant improvement in TSS at the end of phase T (mean change from baseline 6.52; P

Health-related quality of life (HRQOL) was evaluated using the Medical Outcomes Study Short-Form 36 (SF-36), with aggregation into a physical component summary (PCS) or mental component summary (MCS) index; higher scores indicate better HRQOL. Responders also experienced significant improvement from baseline in both PCS (+3.47; P

Patients classified as responders in phase I were randomized for phase II, a double-blind parallel-group 4-week withdrawal study with either placebo (n = 103) or domperidone 20 mg 4 times a day (n = 105). During phase II, both groups experienced deterioration in TSS. The placebo group experienced significantly greater deterioration than the domperidone group (+1.84 placebo versus +0.85 domperidone; between-group difference P = 0.025).41

In phase II, responders receiving placebo demonstrated a significant decline in PCS (1.77; P- 0.20), while those receiving domperidone experienced a statistically insignificant change (+0.65; P = 0.361; between-group P = 0.050). Both groups’ MCS scores were statistically unchanged in phase II.40

Patients were also evaluated by scintigraphy at the outset of the study for gastric emptying rates and classified into delayed gastric emptying (DGE; n – 126; 44%) and normal gastric emptying (NGE) classes; criteria were not reported. No significant difference was seen between NGE and DGE groups in baseline TSS or TSS improvement during phase I, nor in the deterioration of TSS in phase II.41-42

Talley38 has suggested that the two-phase withdrawal design might be suboptimal because of the open-trial design of the first phase. In addition, a straightforward interpretation of the results of the withdrawal phase depends on the assumption that the disease relapses and remits, which may not be the pattern in diabetic gastroparesis. Silvers et al41 acknowledge that potential bias of investigators in assigning total symptom scores at the end of phase I might have been a factor in the deterioration of TSS by the domperidone group in phase II. Talley38 suggests that double-blind, parallel group studies remain the trial design of choice, using validated outcome measures of both symptoms and quality of life.

Gl Symptoms in Parkinson’s

Patients with Parkinson’s disease can experience upper GI symptoms as a result of cerebral degeneration, degeneration of the myenteric plexus and antiparkinsonian medication.*’ Extended domperidone therapy (20 mg 4 times a day, mean follow-up 3 years) has been shown to improve upper GI symptoms and gastric emptying.”’47

Gastroesophageal Reflux Disease

Domperidone has not been shown conclusively to reduce the number of reflux episodes or improve gastroesophageal reflux dis ease (GERD) symptoms. At this time, acid-suppressive agents have become the drugs of choice for GERD.48

Tolerability/Adverse Effects

In one study of 17 patients with documented gastroparesis, oral domperidone 40 to 120 mg/day was used for periods of up to 4 years and was well tolerated.4’4 In the large study by Silvers et al,41 the tolerability profile was similar to placebo. The most common (occurring in as many as 10% to 20% of patients) adverse effects described in most studies are related to increased prolactin levels due to antagonism of D2 receptors in the anterior pituitary, including gynecomastia, galactorrhea, breast tenderness or menstrual irregularities.14- In a double-blind, nonplacebo-controlled, comparison of domperidone and metoclopramide in 93 insulin- dependent patients with diabetes, elicited C]NS effects (somnolence, akathisia, asthenia, anxiety, depression and mental acuity) were fewer and less severe for domperidone.49 case reports describing extrapyramidal symptoms have been summarized elsewhere’ and in general indicate that the risk of developing extrapyramidal side effects is minimal when domperidone is taken orally by adults at recommended doses.

Increased blood pressure and heart rate have been seen in patients treated with oral domperidone 10 mg 3 times a day in conjunction with continuous subcutaneous infusion of apomorphine.'” Parenteral forms of the drug were withdrawn from the market in 1984 due to QT prolongation, ventricular arrhythmia and cardiac arrest in patients receiving high IV doses.3,12 Physiologically relevant concentrations of domperidone have been shown to prolong cardiac repolarization in isolated guinea pighearts, indicating the potential for association with QT prolongation and ventricular arrhythmia clinically.” No clinically important changes in vital signs or electrocardiographic activity were observed in 286 patients using domperidone 20 mg 4 times a day for 4 weeks and in 105 patients who were treated for 8 weeks.41

Summary

Domperidone is a dopamine D2 receptor antagonist with antiemetic properties and localization outside the blood-brain barrier that have made it a useful adjunct in therapy for Parkinson’s disease. There has been a renewed interest in antidopaminergic prokinetic agents since the withdrawal of cisapride, a 5-HT4 agonist, from the market.1 Domperidone continues to be an attractive alternative to metoclopramide because it causes fewer CNS side effects. Patients receiving domperidone or other prokinetic agents for diabetic gastropathy or gastroparesis should also be managing diet, lifestyle and other medications to optimize gastric motility.

References

1. Tonini M, Cippollina L, Poluzzi E et al. Review article: Clinical implications of enteric and central D2 receptor blockade by antidopaminergic gastrointestinal prokinetics. Aliment Pharmacol Ther2QQ4; 18(4): 379-390.

2. Barone JA. Domperidone: Mechanism of action and clinical use. Hosp Pharm 1998; 33(2): 191 -197.

3. Barone JA. Domperidone: A peripherally acting dopamine2- receptor antagonist. Ann Pharmacothcr 1999; 33(4): 429 440.

4. Brogden RN, Carmine AA, Heel RC et al. Domperidone: A review of its pharmacological activity, pharmacokinetics and therapeutic efficacy in the symptomatic treatment of chronic dyspepsia and as an antiemetic. Drugs 1982; 24(5): 360-400.

5. Albright L Domperidone in lactation: Use as a galactagogue. IJPC2004; 8(5): 329-335.

6. Gabay MP. Galactagogues: Medications that induce lactation. J Hum Lact 2002;18(3):274-279.

7. Henderson A. Domperidone: Discovering new choices for lactating mothers. AWHONNLifelines2003; 7(1): 54-60.

8. Gralla RJ. Antiemetic therapy. In: Bast RC, Kufe DW, Pollock RE et al, eds. Cancer Medicine. 5th ed. Hamilton, Ontario: B.C. Decker, Inc.; 2000.

9. [No author listed.] Nausea and vomiting-An introduction. 2004. Amdipharmpic. Available at: www.nauseaandvomiting.co.uk/ NAVRES001- 2-NandV-general.htm. Accessed June 14, 2004.

10.[No author listed.] Parkinson’s disease: An overvie\w. 2002- 2004. Available at: www.pdf. org/AboutPD/. Accessed June 30, 2004.

11 .[No author listed.] Domperidone (Systemic) (electronic version). Greenwood Village, CO: Thomson Micromedex; 2004.

12. Parfitt K, ed. MART/NDALE: The Complete Drug Reference. 32nd ed. London: Pharmaceutical Press; 1999:1190-1191.

13.Parkes JD. Domperidone and Parkinson’s disease. Clin Neuropharmacol 1986;9(6):517-532.

14.Agid Y, Pollak P, Bonnet AM et al. Bromocriptine associated with a peripheral dopamine blocking agent in treatment of Parkinson’s disease. Lancet 1979:1(8116): 570-572.

15.Factor SA. Literature review: Intermittent subcutaneous apomorphine therapy in Parkinson’s disease. Neurology 2004: 62(6 Suppl 4): S12-S17.

16.Bowron A. Practical considerations in the use of apomorphine injectable. Neurology 2004: 62(6 Suppl 4): S32-S36.

17.Hagell P, Odin P Apomorphine in the treatment of Parkinson’s disease. JNeurosci Nurs2001; 33(1): 21-34, 37-38.

18.Pietz K, Hagell P, Odin P. Subcutaneous apomorphine in late stage Parkinson’s disease: A long term follow up. J Neurol Neurosurg Psychiatry 1998:65(5):709-716.

19.Corsini GU, Del Zompo M, Gessa GL et al. Therapeutic efficacy of apomorphine combined with an extracerebral inhibitor of dopamine receptors in Parkinson’s disease. Lancet 1979: 1(8123): 954-956.

20. Jansen PA, Herings RM, Samson MM et al. Quick titration of pergolide in cotreatment with domperidone is safe and effective. Clin Neuropharmacol 2001:24(31:177-180.

21.Hobson DE, Fourcher E, Martin WR. Ropinirole and pramipexole, the new agonists. Can J Neurol Sd 1999: Suppl 2: S27-S33.

22.Fujii Y, Saitoh Y, Tanaka H et al. Prophylactic oral antiemetics for preventing postoperative nausea and vomiting: Granisetron versus domperidone. Anesth Analg 1998: 87(6): 404-1407.

23. Gralla RJ, Osoba D, Kris MG et al. Recommendations for the use of antiemetics: Evidence-based, clinical practice guidelines. J Clin Oncol 1999:17(9):2971-2994.

24.[No author listed.] Chemotherapy- and radiotherapy-induced nausea and vomiting. Amdipharm2QQ4. Available at: www.nauseaandvomiting. co.uk/NAVRES001-5-chemo-radio.htm. Accessed June 14, 2004.

25.Dupuis LL, Nathan PC. Options for the prevention and management of acute chemotherapy-induced nausea and vomiting in children. Paediatr Drugs 2003: 5(9): 597-613.

26.Smith DS, Ferris CD. Current concepts in diabetic gastroparesis. Drugs 2003:63(131:1339-1358.

27.Vaughan CJ, Aherne AM, Lane E et al. Identification and regional distribution of the dopamine D(1 A) receptor in the gastrointestinal tract. Am J Physiol Hegul lntegr Comp Physiol 2000: 279(2): R599-R609.

28.Valenzuela JE, Dooley CP. Dopamine antagonists in the upper gastrointestinal tract. Scand J Gastroenterol Suppl 1984: 96:127- 136.

29.Prakash A, Wagstaff AJ. Domperidone. A review of its use in diabetic gastropathy. Drags 1998: 56(3): 429-445.

30.Franzese A, Borrelli O, Corrado G et al. Domperidone is more effective than cisapride in children with diabetic gastroparesis. Aliment Pharmacol Ther2002:16(5): 951 -957.

31.Koch KL, Stern RM, Stewart WR et al. Gastric emptying and gastric myoelectrical activity in patients with diabetic gastroparesis: Effect of long-term domperidone treatment. Am J Gastroenterol 1989: 84(9): 1069.

32.Schuurkes JA, Van Nueten JM. Dose-dependent stimulation of antroduodenal coordination by domperidone via specific dopamine- receptors. Arch Im Pharmacodyn Ther 1982; 256(2): 311-314.

33.Takahashi T, Kurosawa S, Wiley JW et al. Mechanism for the gastrokinetic action of domperidone. In vitro studies in guinea pigs. Gastroenterology 1991 ; 101 (3): 703-710.

34.Kusunoki M, Taniyama K, Tanaka C. Dopamine regulation of [3H]acetylcholine release from guinea-pig stomach. J Pharmacol Exp Ther 1985; 234(31:713-719.

35. Sanger GJ. Effects of metoclopramide and domperidone on cholinergically mediated contractions of human isolated stomach muscle. J Pharm Pharmacol 1985; 37(9): 661 -664.

36. Horowitz M, O’Donovan D, Jones KL et al. Gastric emptying in diabetes: Clinical significance and treatment. Diabet Med2002; 19(3): 177-194.

37.LaI L, Cushenberry L. A review of diabetic gastropathy. 2002; 27(11 ). Available at: www.uspharm acist.com/ index.asp?show=article&page=8_ 998.htm. Accessed June 6, 2004.

38.Talley NJ. Diabetic gastropathy and prokinetics. Am J Gastroenterol2003; 98(2):264-271.

39.Sturm A, Holtmann G, Goebell H et al. Prokinetics in patients with gastroparesis: A systematic analysis. Digestion 1999; 60(5): 422-427.

40.Farup CE, Leidy NK, Murray M et al. Effect of domperidone on the health-related quality of life of patients with symptoms of diabetic gastroparesis. Diabetes Care 1998; 21(10): 1699-1706.

41.Silvers D, Kipnes M, Broadstone Vet al. Domperidone in the management of symptoms of diabetic gastroparesis: Efficacy, tolerability, and quality-of-life outcomes in a multicenter controlled trial. DOM-USA-5 Study Group. CHn 7/)eM998; 20(3): 438- 453.

42.Patterson D, Silvers D, Kipnes M et al. Does gastric emptying status in patients with diabetes influence the effectiveness of domperidone? [Abstract] Gasfroenrerology 1997; 112(4) Supplement: A804.

43.Soykan I, Sarosiek I, McCallum RW. The effect of chronic oral domperidone therapy on gastrointestinal symptoms, gastric emptying, and quality of life in patients with gastroparesis. Am J Gastroenterol 1997; 92(6): 976-980.

44.Dumitrascu DL, Weinbeck M. Domperidone versus metoclopramide in the treatment of diabetic gastroparesis. Am J Gastroenterol 2000; 95(1): 316-317.

45.[No author listed.] Cochrane Upper Gastrointestinal and Pancreatic Diseases Group. Metoclopramide and domperidone in the treatment of gastroparesis (Cochrane Protocol). Abstracts of Cochrane Reviews. The Cochrane Library Issue 4, 2004. Available at: www. cochrane.org/ cochrane/revabstr/UPPERGIAbstractlndex.htm. Accessed December 21, 2004.

46. Jost WH. Gastrointestinal motility problems in patients with Parkinson’s disease. Effects of antiparkinsonian treatment and guidelines for management. Drugs Aging mi; 10(4): 249-258.

47.Soykan I, Sarosiek I, Shifflett J et al. Effect of chronic oral domperidone therapy on gastrointestinal symptoms and gastric emptying in patients with Parkinson’s disease. Mov Disord 1997; 12(6): 952-957.

48.Maton PN. Profile and assessment of GERD pharmacotherapy. Cleve Clin J Med 2003;70Suppl5:S51-S70.

49.Patterson D, Abell T, Rothstein R et al. A double-blind multicenter comparison of domperidone and metoclopramide in the treatment of diabetic patients with symptoms of gastroparesis. XIm J Gastroenterol 1999; 94(5): 1230-1234.

BO.Sigurdardottir GR, Nilsson C, Odin P et al. Cardiovascular effects of domperidone in patients with Parkinson’s disease treated with apomorphine. Acta Neurol Scand 2001 ; 104(2): 92-96.

51.Drolet B, Rousseau G, Daleau P et al. Domperidone should not be considered a no-risk alternative to cisapride in the treatment of gastrointestinal motility disorders. Circulation 2000; 102(16): 1883- 1885.

Lisa M. Albright, PhD

Austin, Texas

Address coirespondence to: Lisa M. Albright, PhD, at lmalbr- @alum.mit.edu

Copyright International Journal of Pharmaceutical Compounding Mar/ Apr 2005

Bamboo Shortage Threatens Pandas in China

SHANGHAI, China (AP) — Giant pandas in western China could be at risk of starvation because the bamboo plants that they eat are beginning to die off in a cycle that happens about every 60 years, the official Xinhua News Agency reported Sunday.

Workers at the Baishuijiang State Nature Preserve in the northwestern province of Gansu plan to monitor the 102 pandas in the preserve for signs of hunger, according to Xinhua.

Threatened pandas will be moved to areas that still have bamboo, with special attention given to older, feeble animals, it said, citing Zhang Kerong, the preserve’s director.

Pandas derive most of their nutrition from arrow bamboo and can starve once the plant enters its dying-off stage. The stage begins when the bamboo forms flowers, after which the pandas refuse to eat it. The bamboo then starts to produce seeds before dying.

Blooming happens about once every 60 years, with a new crop taking 10 years to mature. However, the cycle seems to run along different schedules in different places and an earlier mass die-off of bamboo in the 1980s caused the deaths of about 250 pandas, Xinhua said.

Xinhua said some bamboo also has started blooming in Sichuan and Shaanxi provinces, home to the rest of China’s estimated 1,590 wild pandas.

Flowering bamboo now covers more than 17,300 acres of the 544,000-acre preserve, Xinhua said. It said 22 pandas living in the preserve’s Bikou and Rangshuihe areas were directly threatened with starvation.

Zhang said rangers would patrol for ailing animals and rescue those in need. Local villagers also have been told not to drive away or harm pandas if they enter inhabited areas looking for food.

China regards the panda as an unofficial national mascot, but the animal’s limited diet is just one factor threatening its survival. Panda numbers have declined as its habitat has fallen to farming and development, and the animal’s low fertility rate causes it to reproduce at an agonizingly slow rate.

Chinese zoologists have improved the birth rate of giant pandas in captivity through artificial insemination. The country also has launched a project to clone the animal as a way of boosting its numbers.

Molecule Movie Offers New Twist for Kids

NORTH GREENBUSH, N.Y. (AP) — Old school: sitting under a planetarium dome gazing at a simulated Sagittarius. New school: watching smiling, singing, animated atoms zip around the dome as they journey through a falling snowflake and a stick of chewing gum.

A children’s museum near Albany is debuting something new on its big-domed screen they call a “Molecularium” show. The 20-minute digital animation piece reinterprets the traditional planetarium experience for kids as likely to stick their nose in a Game Boy as a book. The subject isn’t outer space this time, but atomic space. The movie tells the story of an oxygen atom, Oxy, and her nano pals exploring protons and electrons – a sort of science meets Shrek story for the early grades.

“This was as good as Saturday morning cartoons for them, or better,” said Jayne Architzel, executive director of the Children’s Museum of Science and Technology.

The Molecularium’s creators call it a pioneering mix of art and science. But at its heart, the high-octane show is a new answer to a question dogging many educators:

How do you get young kids interested in science?

The idea came from Linda Schadler, professor of materials science at Rensselaer Polytechnic Institute, which designed and developed the show. Schadler, who heads educational outreach for the school’s nanoscale center, wanted a way to explain molecules to kindergartners through third graders and to explain the three states of matter: gas, liquid, solid. What better way to immerse kids in those concepts than the enveloping dome of a planetarium?

An early challenge was translating textbook illustrations of molecules – those complex, twisted chains – into something digestible for young minds. Shekhar Garde, an RPI professor in charge of getting the science right, and director V. Owen Bush knew precisely what they did NOT want to do.

“Most planetarium shows I’ve ever seen there is this God-like voice that’s speaking down to you,” said Bush.

“This. Is. Nep-tune,” Garde intoned.

They decided an animated story would be a better way to reach kids.

Rabbits and ducks as easy to animate. But how do you make oxygen cute? Their answer was Oxy, a long-lashed, squeaky-voiced character who resembles an orange with a face. She and her hydrogen sidekicks Hydro and Hydra (H20, get it?) take thrill rides through different colorful atomic realms.

Atomic characters form molecules by bonding cheek-to-cheek, bounce around and break out in song verses like: “We make yoooouuu! We make all of you!” They also meet Carbon, a Hispanic atom who pronounces his name like Ramon.

It seems to work. Tara Molloy-Grocki, a teacher at nearby Guilderland Elementary School, took a group of second graders to the museum recently, ushering them into the show after a preparatory lesson on molecules at the museum.

“They were engaged and excited,” Molloy-Grocki said. “Carbon was their favorite character.”

It’s a lot of high-tech razzmatazz. But the aim of the film, funded in part by the National Science Foundation, remains every bit as educational as a sober old planetarium show. The animated atoms are essentially a sweetener to give kids a taste for science. Bush called the Molecularium a careful balance of science and magic.

Cindy Workosky of the National Science Teachers Association said that sort of innovative science instruction – she calls them lessons with a “wow factor” – are important to get kids interested in science.

“We need to be building a foundation of science so that they’re prepared when they go to middle school and high school,” said Workosky, whose group represents 55,000 science instructors from grade school to college level.

With the Molecularium show continuing its run at the museum, Garde said there are in discussions with other planetariums to take it on the road.

“Our goal is to take it to as many venues as possible,” he said.

—–

On the Net:

http://www.molecularium.rpi.edu/

Once-a-Month Osteoporosis Pill May Cut Side Effects

Flip the calendar and take your pill.

Such will be the routine for osteoporosis patients who take Boniva, a once-a-month pill approved Friday by the U.S. Food and Drug Administration.

Boniva will be sold by two giant drug makers, Hoffmann-La Roche Inc. of Nutley and GlaxoSmithKline PLC, which say their product will be the first-ever tablet taken monthly for a chronic condition.

Other drugs in Boniva’s class are widely used for preventing and treating osteoporosis – and taken weekly. But they have a somewhat cumbersome regimen. Patients take them first thing in the morning on empty stomachs, must be sitting up when they ingest the pills and cannot lie down or eat for 30 minutes. The drugs can cause side effects such as stomach discomfort or heartburn.

Boniva patients will need to maintain similar regimens. But doctors hope reducing the pill frequency could help patients stay on their medications.

“Once-monthly medicine truly adds a new convenience to their lives that hopefully will let them stay on therapy longer,” said Fern Heinig, Roche’s brand director for Boniva.

Boniva is scheduled to reach pharmacies in the middle of next month. The drug was approved for women – who are afflicted by osteoporosis an estimated four times more often than men – for the treatment of postmenopausal osteoporosis.

“I’m very enthusiastic that we have another treatment option,” said Dr. Bess Dawson-Hughes, president of the National Osteoporosis Foundation. “No single or small group of drugs will be suitable for all patients.”

Osteoporosis is a bone-weakening disease that can progress painlessly and without symptoms until a bone breaks, usually in the hip, spine or wrist. One in two women and one in four men older than 50 will have an osteoporosis-related fracture, according to the National Osteoporosis Foundation.

Theoretically, Dawson-Hughes said, the need to take only 12 Boniva pills a year, compared with 52 for the weeklies, might help patients stay on the new drug or reduce the times they endure side effects. For chronic conditions such as osteoporosis and high cholesterol, some patients tend to lose interest in taking their medicines – in part because the drugs don’t provide the immediate relief the way a medication such as aspirin does for a headache.

Rather, Boniva and competing drugs are designed to be taken for years to cut the risk of bone fractures.

Dawson-Hughes said studies will test whether Boniva patients are more likely to maintain their therapy. She and others worry that because the drug is taken so infrequently, patients might forget it altogether.

“It’s impossible to guess how all of these things will balance out with this new drug, but it’s great to have a new drug to evaluate further along these lines,” said Dawson-Hughes, a professor of medicine at Tufts University.

Heinig said Boniva patients will be able to join a patient- support program and be alerted by postcard, phone call or e-mail to take their pills.

Roche won approval in 2003 for Boniva as a once-a-day option. But the company decided it would not make enough of a dent in a market of once-weekly versions of drugs in the same class – Fosamax from Merck, and Actonel from Sanofi-Aventis and Procter & Gamble.

The companies behind Boniva said the monthly drug showed in a test of 1,600 women that it was at least equivalent to the daily version in increasing bone mineral density after a year. Dr. Ralph Marcus, director of the Osteoporosis Center of North Jersey in Teaneck, said he would like to see more data on Boniva’s ability to reduce hip fractures, because strong data exist on that front for Fosamax and Actonel.

But that said, “some patients will certainly find it more convenient to take medicine once a month,” Marcus said. “And it may be more tolerable, because if side effects occur it may be only once a month instead of once a week.”

Patients must take Boniva when they wake up. They must remain upright and avoid food, drink and other medications for at least 60 minutes.

Boniva’s price will be comparable to other drugs in its class on a monthly basis, Heinig said.

Boniva is expected to be one of the newer drugs leading an explosion in the osteoporosis market. Annual revenues in the United States from such drugs are expected to climb from $3.3 billion in 2003 to $8.6 billion in 2010, according a January report by Frost & Sullivan, a consulting company.

Aside from bisphosphonates such as Fosamax and Actonel, drugs in other classes – such as Eli Lilly’s Evista – are either in development or on the market.

In the future, osteoporosis drugs may be taken even less regularly. Beyond the monthly, Roche expects to hear from the FDA later this year about its intravenous version of Boniva taken every three months. Companies such as Novartis and Amgen are testing osteoporosis drugs that would be administered even less frequently.

***

E-mail: [email protected]

No Slacking Off for the Ultimate Slacker ; Interview With Damien Duff

Tom Humphries on how the winger may seem to be dancing diffidently through Chelsea’s season but has had to work hard for things to be this good

Midday on Tuesday. He slips from a taxi and comes slouching towards reception without fuss or fanfare. His room key is ready and waiting. “Thanks.” And he’s gone. No need for credit cards to be running through machines or for questions about breakfast or parking or alarm calls. He travels light.

On the way to his room he meets Joe Walsh, the kitman. “Don’t call me for dinner, Joe,” he says.

Sleep. He regrets ever having mentioned sleep in an interview some years ago and laughs at the image he has created of himself as some sort of sloe-eyed Eeyore character who dozes his way through the intervals which punctuate his encounters with a football. Still, it has to be said, Damien Duff sleeps a lot. Were sleeping a sport he’d be world class.

He goes to his room and he sleeps. Some time later a trayful of room service attempts to gain entry to that room. It is rebuffed. Too kipped out. “Thanks.”

A couple of hours on and he is up and about, standing in for the official team photograph taken on the sward at the back of the hotel and then off to Malahide for training. Not for the first time it strikes everyone watching that the contact of a football with Damien Duff’s person seems to send a jolting current of electricity through him. When he hasn’t a ball at his feet he looks permanently in repose. Content and restful.

A football brings him to life but not in the same way as the prospect of an interview does. Different sorts of animation. He is a fugitive from attention, constantly fleeing his own fame. You approach him across the lobby, nevertheless. You have authority to act.

“Howya,” he says. “Howya,” you reply, “how are things?” His eyes have narrowed though, pre-empting you.

“We’re not doing this today are we?” he says. If a face could sound an alarm siren Damien Duff’s would be in a full state of emergency now and cars would be pulling over to the side of the road to let him escape at full speed.

“Well, no. We can leave it for a day or two.””Grand,” he says, well pleased by this suggestion. “We’ll do it in Israel then, yeah.”

Visibly relieved, he restores himself to the company of his team- mates. Thursday in Israel! Getting Damien Duff into an interview situation is like getting a kid with a sweet tooth into a dentist’s chair during a world anaesthetics shortage.

“I’ll try but you know what he’s like,” Pat Devlin had said when you called to ask him about the prospect of an interview with his friend, client and protege.

“He’s the worst in the world,” laughed Pat, “a divil.” We do know what he’s like. Divilish, certainly. The best company in the world though, the essence of decency, the antithesis of the spoiled Premiership superstar. Just a young fella who off the field is very backward about coming forward.

Shy. Reticent. Keeps himself to himself in an almost miserly way. Never saw a tape recorder he liked the look of. Brimful of his own humility.

Chelsea had a press day recently for one of their big European nights. Duffer was corralled through the front door and then slipped straight out the back door. A journalist gave pursuit, caught him and wrestled him to the ground like a cowboy roping a steer. He extracted a quote or two. It wasn’t an easy transaction for either party. They both came away feeling a bit dirty.

So, Israel. Another day. Another hotel lobby. He spots you as he emerges from the team bus with his boots in his hand. He smiles but his eyes flicker. Can’t be time for that postponed dental appointment already? Hot damn.

It is though. He has a bite to eat and finally you get him to a seat on the quiet side of a lobby big enough to hold a seven-a-side in. You tell him to relax but he sits lightly on the chair as if leaning back will cause it to break. You tell him that this won’t hurt a bit – but every dentist says that.

You switch the mini-disc machine on. He winces. You haven’t asked him anything yet. You have lots of interesting questions with which to bamboozle him but he needs a warm-up. There is a long pause. Duffer looks at the mini-disc machine. Infernal contraption! Interviewer looks at Duffer. Hmmm. Fifteen years of accumulated interviewing technique. You summon every scintilla of experience and interrogative know-how. You have ways of making him talk. Let the bamboozling begin! Finally you hit him with your best shot.

“So, eh, how are things?” For a second everyone passing through the lobby freezes. Everyone glances across in wonder. Not just Irish fans. Locals too. “How are things?” The most dazzling opening question ever asked.

And to Duffer. What impudence! What derring-do! There was a time when coming on these trips with the Ireland team essentially meant travelling in the wash of Roy Keane. All anyone wanted to know about was Roy Keane, Roy Keane, Roy Keane. Duffer’s pre-eminence is threatening those levels now, however. Chelsea. The Champions League. He’s gone big time. A marquee name in a Broadway production.

And he’s pondering your multi-faceted question. More philosophical inquiry than question really. So. How. Are. Things.

How shall we define things? We’re paused over that one. The opening gambit in what will be a beautifully played chess game.

“Ah, things are grand,” he says cautiously. “Going all right. So many games.” He pauses.

“How are things, yourself.”

Another pause. Gasps in the lobby. Premiership superstars aren’t suppose to ask hacks how things are. You’re instinct is to start again. Ask him the ingenious “how are things” question but with the mini-disc machine switched off. Foreplay.

Get him humming.

It doesn’t take long for a fan to interrupt this breakdown in communication. A middle-aged Israeli woman in a flamboyant red blouse approaches, bearing in her hand a white beer mat. Can he please sign this. “Of course, of course.” He thanks her for asking.

She tells him that he is her daughter’s hero. He blushes madly although he must be used to this sort of thing by now. The Argentine stewardess on the flight to Tel Aviv the previous day had been so besotted by his Dufferesque “cuteness” she had to go public on the matter as we disembarked.

“I hope you win on Saturday,” says the Israeli lady as she backs away, bowing in an oriental manner as she goes.

He smiles and thanks her again.

“You don’t think she meant that, do ya,” he says as she recedes, “about hoping we win?” Who knows? Life is full of sweet mystery. Damien Duff plays football. About once in a blue moon he does interviews. He doesn’t know why. That, by the way, is your second question, the searing follow-up to “how are things?””So, why do you do interviews?”

“Pat rang and asked me would I do it,” he says, treating this like a genuinely interesting line of interrogation. “He caught me at an off moment. I don’t really know why I said I’d do it. But, sure . . . ” he just trails off.

You glance down at your lovely little mini-disc machine, which, optimistically, is set to record these priceless exchanges at half speed, thus giving you three hours of perfectly recorded conversation. Hmmm.

“So,” you say. You’re in Beazer Homes League form today. “Things are good?”

“Ah, yeah. Lots of games. Nothing else up. Just plugging away. It feels like a treadmill at times. I’m not used to playing that many games. I’ve had injuries and all that in the past seasons. This season – touch wood – it’s going well, injury-wise.

“I’ve played an awful lot of games compared to what I would usually play.”

There you go. See. A 58-word answer to your third question. Not Parkie but not quite Alan Partridge either. Up your game a bit and Duffer will play ball.

“Wow,” you gush, girlishly, “so, how many games have you played then?”

For the record, he reckons he’s played 45 games or so this year, so far. By March, between hamstring tweaks and shoulder injuries and all the other mandatory pitstops he’s usually around the20 mark. He enjoys the business of being busy.

“What do you put the lack of injuries down to?”

“I had the operation in the summer and it’s been all strengthening work since then. In the past there’s been a lot of things like hamstrings and what have ya. I put it down to the training at the club and all the work the gaffer does. I benefit from that really.”

This is the essential charm of Damien Duff. He is at the centre of a media storm and his hair isn’t even blowing in the wind. Chelsea are the story of the football year, a constant, unceasing drama which has changed the customary narrative of Premiership seasons, relegating the houses of Highbury and Old Trafford to the status of weary sub-plots. Mourinho is the lead player, a bespoke genius who acts as a lightning rod in the tempest of tabloid fury and speculation.

To Damien Duff it’s just Chelsea and the gaffer. It’s not even that. It’s just football. It could be Lourdes Celtic. Or St Kevin’s. Or Blackburn reserves. What matters most is when it’s Saturday afternoon and the clock has slipped past 4.30 and you’ve the ball at your feet and two men to beat. It’s all still as simple, as beautiful and as mesmerising as that. If that’s all you capture on the mini-disc machine, so be it. That’s the man.

“It doesn’t intrude at all,” he says when you ask him if the maelstrom of publicity around the club ever gets inside his head. “The tabloids are after the gaffer, I suppose. He takes the hits for everyone. I stay out of it. I always have done.

“Obviously Chelsea is a different world to Blackburn but for me this is the first proper interview I’ve done in God knows how long. I can’t remember the last time I sat down with a journalist. I know Chelsea is different, especially this year, but I’m just the same old me.”

He has no part in the Premiership Babylon. He doesn’t treat with agents or spivs or Ferrari salesmen or Flash Harrys. He doesn’t have any scores to settle, women to roast or bling to wear. You can skip to the end of this interview safe in the knowledge that there is no fodder for a tabloid headline ANGRY DUFF SLAMS XXXX!

“I don’t have time for it,” he says, apologetically. “I don’t know. I’ve no time for that stuff. I’d say I just come across as an ignoramus to those kind of people. Agents and shit. I live out near the new training ground. We all live about 40 minutes outside London. I don’t go into London that much. I don’t be near that world to be honest. I have the quiet life.”

Thus it is that he has no tales to tell other than his own, which he keeps sparse and private. He is immune to the madness but he has seen the way it works, how a careless word sparks a tinderbox. So if he speaks well of Chelsea, he makes a detour to point out that by speaking well of Chelsea he is not deriding Blackburn, which he loved and adored.

The Chelsea he joined are metamorphosing into a world-class outfit with world-class facilities. It’s a world away from Lancashire but he would never disparage.

He knows other things too. Frailty, thy name is football. He is lauded and worshipped now, part of the only genuine and functioning side of galacticos the world game has, but at the start of the season he was looking down both barrels. Failure and injury were about to blow him away.

Hard times. When you are impervious to everything but football and family it is no consolation to be a millionaire and to be sitting at home in your millionaire’s house on a Saturday afternoon when there are men out there on pitches up and down England living your dream. You pick up the Sundays and you notice that hardly anybody is lamenting your absence. It struck him then that it could all be taken away just like that. Football never needs you as much as you need it.

“It was hard at first this season,” he says. He’s slightly animated by the thought of it but careful not to be seen to make too big a deal of it. “I was coming back from my shoulder injury and I thought I was fit but I don’t think the gaffer thought I was fit. Either that or he thought I was fit but he thought I wasn’t good enough. Obviously it wasn’t nice not getting into the squads, or what have you. That was hard. That hit me, all right. I just kept working, working away really hard.”

And that was it. He didn’t rub a water bottle and have a genie suddenly pop out. There was no Damascus moment. People close to him, and other players at the club, said he should go and speak to Jose Mourinho, to ask him what was happening.

Duffer and Jose Mourinho are at the opposite ends of the personality spectrum, however.

“Everyone was saying to me that I should go and have a chat with him. That’s never been me either. I wanted to just keep working hard. Eventually he pulled me aside He wouldn’t pull you aside that much. I’ve only really had that one chat one-on-one with him. Just that once back at the start of the season. “

“What did he say?”

“We just had a chat. A couple of weeks later I was back in the team.”

“So, what did he say?”

“I wouldn’t like to mention it.”

At this point you want to weep and say puh-lease mention it, puh- lease, but politely Duffer’s face tells you the conversation was private. So, instead you make a stab at guessing. You put yourself in Mourinho’s expensive shoes. Limited English.

Bright man. And Duffer. Bright man too. Better English but not willing to use it.

“Keep working hard? Did he say that?””Not exactly.”

“You’re on the way out? Did he say that?”

“Can’t say really.”

“Did you come out of the chat feeling happier?”

“Not happier. No.”

“Worried?”

“A bit of that. Yeah.”

“Panicky?”

“More determined. I decided I wanted to stay at Chelsea as long as I can. Anywhere else will be a step down. It would break my heart to know I was leaving and going down. I’ll just keep working hard. I knew after that I was playing for my future. I was running my heart out.”

“Playing for your future?”

“I still think that now. Every day, even in training. I look at the resources Chelsea have. There’s no slacking off. That’s the way I’ve been brought up by the family.”

His restoration to the team marked the end of a long fallow period. He tells the story of his Tuesday night in Aldershot last year, playing for Chelsea reserves against West Ham reserves on a badly ploughed hell-hole of a pitch. He was miserable.

He was playing poorly. He’d touched the ball three times in the 40 minutes he had been on. None of the three touches had excited the man and his dog who together made up the attendance.

Then a local newspaper reporter asked him what he thought of the big news of the day. Chelsea had signed Arjen Robben. The Dutch Duffer. Only younger. And of course Dutch. If a team signed Quasimodo and he was Dutch it would seem glamorous.

“Even before they signed Arjen I was at the lowest point in my career. I was at Chelsea, had just gone for a lot of money and was out for God knows how long, three or four months with my shoulder. Then that night playing on that shitty pitch in Aldershot I did a little interview afterwards and I said it was a kick in the teeth. I wasn’t in the best of moods.”

It’s not as if he went out and bought an Uzi and went crazy in some little town in Surrey but he seems to genuinely regret letting his feelings slip.

“People have used that quote a lot,” he says, shaking his head.

His resurrection, his emergence from the slough of quiet despair, has been a model for our crazy times, the sort of thing which brings you back to the faith. He didn’t call an agent or the tabloids or the off-licence. He just worked. He accepted playing on the other side of the field. He accepted he’d have to do whatever it took to be in the team. Uncomplainingly. Happily.

When his shoulder got better he submitted to a weights regime, which he sticks to zealously. At the start of this season Arjen Robben was injured. Damien Duff still wasn’t getting in the squad.

“I was sitting at home on a Saturday watching Soccer Saturday. I was being a real t*** with my family and friends and girlfriend. I suppose that’s what football means to me. “

Now he’s back. In love more than ever. He loves the season Chelsea are having. He loves the life he lives, lives the life he loves. Simple. He even loves thegaffer.

“We all love him. He’s the man. He’s the best manager around, in my view. He can be very funny. Even the Liverpool thing, he got slaughtered in the press for that. It was a bit of crack. I thought it was funny myself. Not being biased but people go overboard. It’s funny to sit back and watch it.”

He talks with cautious enthusiasm (or rather, enthusiastic caution) about the night against Barcelona in the Champions League, how himself and the lads knew within five, ten minutes of the game in the Nou Camp how the return would pan out.

And that brief feeling of footballing omnipotence, that fleeting but perfect moment when Chelsea went three up in 20-something minutes and Duffer had everyone close to home sitting in the stands and it was the stuff you dream about when you’re a young fella scuffing about in Ballyboden.

Then as if he’s forgotten himself for a moment he says the days which gave him the greatest satisfaction were those games when he worked his way back into the side and they were scratching out workmanlike results in places like Ewood Park.

“Just showed that we weren’t all flash. That we could do the graft too.”

That’s the motif running through it all. The benefits of work, patience and humility. The mini-disc never fills.

A man from the People’s Republic of Roy interrupts with a request for the signing of jerseys. Damien Duff says he’ll come across the lobby and sign the garments in a minute. It would be too bad- mannered to interrupt the flagging interview but too flash to refuse to sign.

This week back in the beloved green jersey he sits as usual at the heart of the squad. Beloved. He was 26 recently, metamorphosing into a senior player, yet there is that lingering sense about him. The team are there to protect him.

You ask him if he has more to say these days at team meetings. He is emphatic on this. “No! I don’t say anything. I know I’m 26 now. A bit old to be considered ‘potential’. There’s more responsibility, I suppose. I’m getting close to being put on the old team when we play old v young at training. I don’t talk at meetings.

“You don’t get a word in with Brian anyway. I just can’t sit still at team meetings. I fidget. Same as ever.”

These past few trips he’s fallen into a card school with Robbie Keane and a couple of other lads. Somehow you don’t see Duffer as one of the world’s most devious poker players. Fortunately for his bank manager, neither does he.

“The first two days this week haven’t gone well. I’ll have the next few days in bed with the DVDs. Cheaper. “

Tonight he expects the lads will give it socks. He would be disappointed with a draw. His passion doesn’t allow for anything but absolute fulfilment.

“We’ve watched the Israelis a lot on DVD. It’s a massive game for us both but with the quality we have, we need to come in here and win.”

There you have it.

The pay-off.

Cocksure Duff Slams Israeli Pretenders!

“We’re Quality” Rails Goldenboy Duff . . . You feel grubby and soiled for your part in having elicited such intemperate words from such a quiet, decent guy.

Post-quotal, Duffer leans back in the chair for the first time. He’s not spent though.

“Of course they’ve a right to say the same thing and you have to respect that,” he adds.

The mini-disc recorder winces. Switches itself off.

So to does Duffer, saving himself again for when Saturday comes.

Starvation is Not Painful, Experts Say

After suffering through cancer, the middle-age woman decided her illness was too much to bear. Everything she ate, she painfully vomited back up. The prospect of surgery and a colostomy bag held no appeal.

And so, against the advice of her doctors, the patient decided to stop eating and drinking.

Over the next 40 days in 1993, Dr. Robert Sullivan of Duke University Medical Center observed her gradual decline, providing one of the most detailed clinical accounts of starvation and dehydration.

Instead of feeling pain, the patient experienced the characteristic sense of euphoria that accompanies a complete lack of food and water. She was cogent for weeks, chatting with her caregivers in the nursing home and writing letters to family and friends. As her organs finally failed, she slipped painlessly into a coma and died.

In the evolving saga of Terri Schiavo, the prospect of the 41- year-old Florida woman suffering a slow and painful death from starvation has been a galvanizing force.

But medical experts say going without food and water in the last days and weeks of life is as natural as death itself. The body is equipped with its own resources to adjust to death, they say.

In fact, eating and drinking during severe illness can be painful because of the demands it places on weakened organs.

“What my patients have told me over the last 25 years is that when they stop eating and drinking, there’s nothing unpleasant about it — in fact it can be quite blissful and euphoric,” said Dr. Perry G. Fine, vice president of medical affairs at the National Hospice and Palliative Care Organization in Arlington, Va. “It’s a very smooth, graceful and elegant way to go.”

Schiavo, who hasn’t had any food or water since Friday, has been in a persistent vegetative state for 15 years that makes it impossible for her brain to recognize pain, doctors say.

“Her reflexes with respect to thirst or hunger are as broken as her ability to think thoughts or dream dreams or do anything a normal, healthy brain does,” Fine said.

But even if her brain were functioning normally and she were aware of her condition, she would be comfortable, doctors say.

“The word starve’ is so emotionally loaded,” Fine said. “People equate that with the hunger pains they feel or the thirst they feel after a long, hot day of hiking. To jump from that to a person who has an end-stage illness is a gigantic leap.”

Contrary to the visceral fears of humans, death by starvation is the norm in nature — and the body is prepared for it.

“The cessation of eating and drinking is the dominant way that mammals die,” said Dr. Ira Byock, director of palliative medicine at Dartmouth Hitchcock Medical Center in New Hampshire. “It is a very gentle way that nature has provided for animals to leave this life.”

In a 2003 study published in the New England Journal of Medicine, 102 hospice nurses caring for terminally ill patients who refused food and drink described their patients’ final days as peaceful, with less pain and suffering than those who had elected to die through physician-assisted suicide.

The average rating given by the nurses for the patients’ quality of death was an 8 on a scale where 9 represented a “very good death” and 0 was a “very bad death.”

Patients deprived of food and water will die of dehydration rather than starvation, unless they succumb to their underlying illness first.

Without fluids, the body loses its ability to maintain the proper balance of potassium, sodium, calcium and other electrolytes in the bloodstream and inside cells.

The kidneys react to the fluid shortage by conserving as many bodily liquids as possible.

The brain, which relies on chemical signals to function properly, begins to deteriorate. So do the heart and other muscles, causing patients to feel tired and lethargic.

“Everything in the body is geared toward trying to maintain that normal balance,” Fine said. “The body will do everything it can to maintain this balance if it’s working well.”

Meanwhile, the body begins mining its stores of fat and muscle to get the carbohydrates and proteins it needs to make energy.

“If you mine too many proteins in the heart, it gets unstable,” Sullivan said. That can give rise to an irregular heartbeat, which can cause the patient to die of cardiac arrest. Or, if the muscles in the chest wall become weak, the patient can end up with pneumonia, he said.

Patients already weakened by disease begin feeling the impact after a few days, Fine said.

They eventually descend into a coma and finally death. The entire process usually takes one to two weeks, although a patient who is otherwise healthy — such as Schiavo — could hold on much longer.

Throughout the process, the body strives to suppress the normal feelings of pain associated with deprivation.

That pain of hunger is only felt by those who subsist on small amounts of food and water — victims of famine, for instance, or concentration-camp inmates. They become ravenous as their bodies crave more fuel, said Sullivan, a senior fellow at Duke’s Center for the Study of Aging.

After 24 hours without any food, “the body goes into a different mode and you’re not hungry anymore,” he said. “Total starvation is not painful or uncomfortable at all. When we were hunting rabbits millions of years ago, we had to have a back-up mode because we didn’t always get a rabbit. You can’t go hunting if you’re hungry.”

After a few days without food, chemicals known as ketones build up in the blood. These chemicals cause a mild euphoria that serves as a natural anesthetic.

The weakening brain releases a surge of feel-good hormones called endorphins.

Doctors also have a host of treatments to ameliorate acute problems, such as sprays and swabs to moisten dry mouths and creams to moisturize flaky skin.

They can also administer morphine or other powerful painkillers.

Sullivan said doctors are likely to give some to Schiavo.

Why a Critical Path By Any Other Name Would Smell Less Sweet? Towards a Holistic Approach to Pert/Cpm

ABSTRACT

To maximize the potential of Critical Chain (CC) to enrich project management practice, I discuss Eliyahu Goldratt’s work in the context of his entrepreneurial career. I show that PERT/CPM had been an instance of Goldratt’s “Theory of Constraints” (TOC) before Goldratt had articulated it. I also highlight errors and questionable recommendations he made. Nonetheless, CC provides a more holistic approach than the typical practice before. I (1) discuss CC and TOC, including strengths and weaknesses, in the relevant context; (2) provide earlier sources for the major so- called Goldratt innovations; (3) identify opportunities for immediate improvement and future research highlighted by Goldratt’s work.

Keywords: PERT/CPM; Critical Chain; Theory of Constraints; Project scheduling

2005 by the Project Management Institute

Vol. 36. No. 1, 27-36, ISSN 8756-9728/03

“What’s in a name? That which we call a rose by any other name would smell as sweet.” W. Shakespeare

1. Introduction and Management by Constraints

In 1988, Eliyahu M. Goldratt, already well known for his commercially successful software Optimized Production Technology (OPT), and already a very successful author (Goldratt & Cox, 1984), articulated what some call a “radically new” approach to managing the flow of materials in factories: the so-called “Theory of Constraints” (TOC) (Goldratt, 1988). In this paper, we’ll use a more fitting term: “Management by Constraints” (MBC) (Ronen & Starr, 1990), since TOC is not an actual theory. MBC replaced a former articulation of Goldratt for the same purpose, which had essentially summarized just-in-time (JIT) principles, but with an additional wrinkle that suggested focusing on the bottleneck (BN) resource- which, under JIT, is partly implicit and partly redundant. MBC, which had emphasized the focusing aspect, could no longer be dismissed as a copy of JIT. At the time, many U.S. managers were keen to adopt JIT benefits, but some of them were less keen to admit the Japanese could teach the U.S. anything. Consequently, Goldratt’s contribution was greeted enthusiastically as radically new and better than JIT. Later, utilizing the more general aspect of MBC, it was touted as the correct approach to the management of everything under the sun. It is based on six steps (Ronen & Starr, 1990), with the first one, which we denote by the index 0, absent in the original:

0. Select an objective function and decide how to measure it.

1. Identify the binding constraints that limit our ability to improve the objective function

2. Manage the binding constraints to improve the objective function maximally. (I am not quoting Goldratt verbatim. His version was “Decide how to exploit the constraint,” but I assume he had not intended to restrict this to planning only.)

3. Subordinate everything else to the needs of the binding constraints.

4. Improve the binding constraints.

5. Return to Step 1, since the binding constraints may have changed after Step 4.

MBC enjoyed a tremendously favorable reception by practitioners. Many felt-perhaps with good reason-that no academic had ever explained these principles so clearly and succinctly. As for academics, some of them joined in, and the ideas-usually with full credit given to Goldratt-found their way into many Production & Operations Management (POM) texts, and certainly influenced academic teaching and research. There is no denying that Goldratt is bright and has a genius for selling ideas. Also, although MBC and earlier teachings mainly involve packaging of old ideas, it is certainly a clever packaging. Nonetheless, others felt that there was nothing essentially new in MBC and that much of it is too simplistic or wrong. The fact that Goldratt had made unfounded claims of optimality with respect to the OPT software long before articulating MBC may have contributed to this negative reception. OPT did not deliver everything it was supposed to deliver. By chance, I know of an example where top management bought the product but the schedules it generated were never actually used on the shop floor. Top management was not informed of this, and it was ultimately revealed when they tried to force it upon a company they eventually acquired. In other words, the software was used as a regular material requirements planning (MRP) system. Possibly, other implementations that were reported as successes never involved the use of the OPT module itself (although that was the central module used to promote the package). Another client, MARS, won a court battle against Creative Output (CO), Goldratt’s company (of which he has since divested himself). The judge set precedence by ordering CO to reveal the source code to MARS, so they could complete their proof that the package could not deliver what was promised (Wilkins, 1984). Eventually, Goldratt stopped making optimality claims with respect to OPT. Goldratt (1988) points out weaknesses of OPT and, indeed, his subsequent software package-DISASTER(TM)-is very different (Simons & Simpson, 1997). Pinedo (1997), inter alia, comments on the success of the OPT package in the marketplace. Furthermore, careful study suggests that Goldratt’s teachings are indeed too simplistic and not always correct (as I discuss in more detail later).

Since both Goldratt’s supporters and opponents have valid arguments, one might expect a consensus to emerge with respect to the correct ideas and the system that connects them. Nevertheless, this has not yet happened. There are myriad academic papers that treat Goldratt as an “academic giant,” and seek a good foothold on his shoulders. This does not mean, however, that academia as a whole admires Goldratt. Understandably, detractors rarely mention Goldratt in writing (and their point of view is usually only expressed verbally), but the existence of this point of view cannot be denied. Last, but not least, papers that seek to study the good and the bad together-exactly what we need! -are rare. Refreshing exceptions are Herroelen and Leus (2001), which is also a rich source of illuminating references (some of which are repeated in this paper); Herroelen, Leus, and Demeulemeester (2002); and Raz, Barnes and Dvir (2003). Also, Erenguc, Tirupati and Woodruff (1997) edited a special issue on capacity-constrained planning and scheduling, most of which is devoted to a balanced discussion of Goldratt’s contribution to scheduling software, including commentary from leading academics. McKay and Morton’s 1998 review of Goldratt’s book, Critical Chain, is another balanced source.

Why is the balanced approach the exception and not the rule? Arguably, the reason is that Goldratt’s personality influences the academic debate. Thus, even Goldratt’s true contributions are denied or ignored by some of his detractors, while many supporters refuse to admit that Goldratt’s teachings are often misleading or erroneous. This state of affairs is not conducive to the proper discovery and evolution of the academic truth. Personally, I believe that a necessary condition to get out of this quagmire is to “call a spade a spade” and discuss Goldratt’s work without pretending that the discussion is totally separated from his personality. (I apologize for using the first person singular while doing so, but the alternative is awkward-the paper reflects my personal take of the “Goldratt phenomenon” and much of the “evidence” is from my personal experience.) Another purpose is to list some of the relevant sources that precede Goldratt. Evidently, Goldratt had made a decision not to provide this academic service himself (and since he is not an academic, but an entrepreneur, why should he?). I hope that this list will help those of us who do not wish to be labeled as “Goldratt disciples” teach and research the issues as any other academic issue, without feeling obligated to pay undue homage to him.

Perhaps the most glaring case of misplaced credit is revealed by Goldratt’s venture into the field of project management. Wittingly or unwittingly, Goldratt had used old and well-known lessons from the project management field to develop MBC. A few years later, he turned around and reapplied them to projects, but, instead of saying so, he effectively claimed that he was applying MBC to projects for the first time! Section 2 is devoted to this issue, and demonstrates that the combination of project evaluation & review technique and the critical path method (PERT/CPM or CPM, in short) had always been an instance of MBC. (For convenience, however, I will reserve the term “MBC” for the manufacturing environment.) Section 3 asks some theoretical questions associated with MBC. It is interesting to see that they are not problematic in the project environment (which raises the question to what extent it has been appropriate to adapt CPM to MBC so faithfully). Section 4 lists some academic sources for the main so-called “innovations” in Critical Chain (CC), and discusses Soviet roots of the pivotal MBC focusing idea. Section 5 discusses the practical state of the art that gave Goldratt the opportunity to sell CC as something different from CPM. Nonetheless, Goldratt’s work can (and should) be a basis for major improvements in project management practice. Section 6 proposes a more holistic approach to project management, including steps we can take in the short- and long-term. Section 7 is the conclusi\on.

2. PERT/CPM as a Seminal MBC Model

Putting focusing aside for a while, it is relatively well known that mathematical programming principles and models (e.g., linear programming [LP]) supersede MBC (Ronen & Starr, 1990). For example, the analysis of opportunity costs and duality theory precedes Goldratt’s observations about the fallacies of traditional accounting and provides a more correct view of the value of time lost on various constraints. And the newsboy model provides a better approach to buffer setting (it may not be necessary to use models for this, however). The focusing aspect, however, seems to give MBC an edge that makes it look different. Here, we will see that this focusing aspect was not invented by Goldratt. In section 4, we discuss Soviet roots for this idea that go back to the early communist era, but it also has a very direct Western origin when we think about project management. The complete MBC structure, along with its focusing features, is well known and has been well known since Goldratt’s childhood. It is simply PERT/CPM! The isomorphism between CPM and MBC is straightforward-to wit, here are the six steps of MBC with project-specific comments added, mostly in bold. I restrict the bold statements to what I remember having been taught as an MBA student in the mid-1970s. (I remember the lessons about LP and cost accounting from the same period.)

0. Select an objective function and decide how to measure it. (The objective is to finish the full scope of a project as soon as possible and within the budget. It is measured by professionals, customers, and calendars.)

1. Identify the binding constraints. (Identify the critical path. This includes the effects of any constrained resources.)

2. Manage the binding constraints. (Focus attention on the critical path to make sure that no time is wasted there. This includes encouraging and utilizing earliness in any critical activity.)

3. Subordinate everything else to the needs of the binding constraints. (The non-critical activities must be started early enough to control the risk that these activities will delay the project. An excessively conservative [but not unusual] implementation of this principle involves starting all activities as early as possible. In addition, the critical path can be used to decide what the project manager needs to pay attention to personally and what can be delegated, which makes it also a rational basis for hierarchical management of projects.)

4. Improve the binding constraints. (Reduce the length of the critical path by crashing and other time-saving methods, where expedient, as long as it is cost-effective.)

5. Return to Step 1, since the binding constraints may have changed after Step 4. (During crashing, the critical path may change, so crashing involves focusing on the evolving critical path iteratively. Even more important than this planning aspect is the control aspect. Due to exogenous developments and chance events, the critical path may change during the project execution. Therefore, it is necessary to manage the future project activities with a focus on the updated critical path. This is equivalent to returning to Step 1. Of course, similar observations about the execution and control aspect apply with respect to flow of materials in a factory.)

Never having met the man, I do not know for sure whether Goldratt was aware of this isomorphism when he articulated MBC. (Indeed, he may have acted on subconscious information.) However, at least to the extent we treat his work as an academic source, he certainly should have been aware. Therefore, changing the name “critical path” to “critical chain” is not “a rose by any other name [that] would smell as sweet.”

3. MBC-Some Questions

CPM starts with identifying the critical path; MBC starts with identifying the binding constraint(s) (i.e., the bottleneck must be known before one can proceed further). There is no dispute that focusing on binding constraints is useful-it may be obvious, but that’s a different matter. Nonetheless, it is not clear at all that we should start by identifying them or that we should schedule production by them. In a factory, a strong case can be made that it is enough to limit the total work in progress (WIP) effectively (Hopp & Spearman, 2001) and avoid large transfer lots (Trietsch & Baker, 1993). The limiting resources may even shift with time without creating major scheduling problems. The (IT system provides a very well-known example and achieves smooth operations without wasting bottleneck capacity and without necessarily identifying the bottlenecks explicitly; in fact, even Goldratt and Fox (1986, pp. 13, 89, 95) recognized JIT’s leadership. A related approach is constant WIP (CONWIP). Hopp and Spearman (2001) and Trietsch (in press-b) explain, in detail, the advantages of CONWIP over Coldratt’s drum-buffer-rope (DBR). Essentially, DBR aims to maintain a constant WIP in the system upstream of the bottleneck resources without attempting to control the WIP downstream from the bottleneck, while CONWIP covers the whole system. If the bottleneck is stable, CONWIP and DBR perform almost equivalently, with perhaps a very small throughput advantage to DBR, at the cost of increased fluctuations risk. But, since bottlenecks should never be trusted to be stable, CONWIP has the major advantage that it will work equally well with any bottleneck, like JIT.

One additional point: although CONWIP is formally defined for flow shops, it is straightforward enough to extend to more complex factories. Basically, the idea is simple: Control the total WIP by a tight feedback loop. Do not induct new work before selling (or scrapping) the same amount. Over time, adjust the WIP cap to get the best performance and to help identify important constraints and improvement opportunities (as in “exposing the rocks” under JIT).

In a make-to-order environment, the system inducts new work if there is a queue of orders and the WIP cap allows, but, when there is no queue, the total WIP in the factory is decreased automatically. Therefore, the system may be called CAPWIP-for capacitated WIP. Together with lot streaming (i.e., using small transfer lots), CAPWIP may be considered a special case of JIT.

As under JIT, it is easy to identify persistent or recurrent bottlenecks under CAPWIP-they are the ones that collect persistent or recurrent machine queues. Other important queues await assembly parts from the bottleneck and we refer to them as assembly queues. (Sometimes, waiting space is the bottleneck; in this case, the analysis is slightly more complex, but the same situation can happen under DBR, and it is not very difficult to identify the true cause in such cases.) In conclusion, CAPWIP does not require identifying the bottleneck in advance, but does help identify bottlenecks and focus on their improvement. It is also relatively easy to implement- certainly easier than DBR, if only because the bottleneck does not have to be identified in advance.

The CAPWIP idea is obvious to anybody who is familiar with DBR, because it can be described as a special case of DBR, with the market holding the drum. This raises a question: why didn’t Goldratt choose CAPWIP? As it happens, in the early 1990s, I consulted for two managers who were using CAPWIP routinely, and both of them were Goldratt Associates. I also had a meeting with a third manager, an avid client of the Goldratt Institute, and he too used CAPWIP. According to this third manager, by then Goldratt had added CAPWIP to DBR-although his books don’t say so. Indeed, one can interpret the following quote from Goldratt (1988, p. 454) in this light: “the OPT BRAIN contains a crude mechanism to prevent excess build ahead.” This would make perfect sense because, otherwise, the shifting bottleneck phenomenon can cause uncontrolled WIP buildup. The two plants managed by these Goldratt Associates were large machine shops, where CAPWIP is by far the easiest practical way to control the total WIP. The third-in the electronics industry-had more options that could have been considered, including DBR, but management still selected CAPWIP. Why, then, did Goldratt not adopt CAPWIP from the start? Why didn’t he adopt it completely and openly later? From an academic point of view, this decision should have been explicitly made and explained.

The consulting I had done with the two Goldratt associates consisted of improving bottleneck capacities (Step 4), by using setup reduction methods and similar improvements (Shingo, 1988). Of course, we first had to identify the bottlenecks. The Race (Goldratt & Fox, 1986) explains how to do this as follows:

A capacity constraint manifests itself in all of the major business issues. An analysis of the major business issues can be used to identify the capacity constraint resources (CCR’s). (p. 107)

In both cases, however, none of us considered using Goldratt’s solution, and I doubt we could have done it had we wanted to. The organization to which the shops belong is huge and its business issues could not be used to pinpoint specific machines in one job shop. At best, such analysis could point to the facility to which the shop belonged as an organizational bottleneck, but that would not solve our problem. A more localized analysis was not a reasonable option either because it would still require vast amounts of reliable data, which was simply not available. Therefore, we selected a less sophisticated approach-observation of queues. The idea is well known from JIT, and was also endorsed by Goldratt in The Race (Goldratt & Fox, 1986, p. 129). An academic validation of this idea is included in Lawrence and Buss (1994). This brings us to the issue of Goldratt’s careful analysis where to place buffers in a factory. The following quote from The Race explains how important it is to plan protection carefully and proactively:

Concentrate protection not at the origin of dis\turbance, but before critical operations. Inventory of the right parts in the right quantities at the right times in front of the right operations gives high protection. Inventory everywhere else is destructive. (p. 113)

Again, the way to identify bottlenecks is through observation of queues, although sometimes they are intangible (e.g., lost orders) or removed from their true source (e.g., assembly queues or queues ahead of blocked machines that should be assigned to downstream resources). It follows that these queues automatically protect the resources that have to be protected. There is no need to plan the exact location of the buffers any more than there is a need to instruct the sun to rise in the east. Again, one must ask what motivated Goldratt’s recommendations. Is it an academic issue? A practical issue? Both? None? Should academics endorse it? Should practitioners pay for the required analysis?

4. Critical Chain-How Much is Really New?

To return to CC, Goldratt’s followers and some others, perhaps, believe that there are radical differences between CC and CPM. There are four main points often raised:

1) The use of resource constraints instead of resource leveling.

2) Abolishing due dates to manage critical (and other individual) activities.

3) The use of project buffers and feeding buffers.

4) The importance of avoiding multi-tasking.

In this section, we will discuss these issues, mostly in terms of their origins. In the next section, we’ll discuss them in terms of the “state of the art in teaching and practicing project management” until the 1990s. The limit of the 1990s is because contemporary, respectable project management texts cite Goldratt’s CC as the source of at least one of the very same ideas we are discussing here. Finally, since this section is focused on the academic sources of Goldratt’s work, it also includes a brief discussion of what is perhaps the earliest source of the bottleneck-focusing idea that is so pivotal in MBC. Of course, CPM itself is another such source.

Resource-Constraint Issues in the Academic Literature

Academic publications addressed this issue since the early stages of the PERT/CPM development. Wiest (1964) identified what he termed “the critical sequence” as a critical path that takes into account resource dependencies. Good POM and operations research (OR) texts always stressed the need to consider limited resources and their implications on the critical path (e.g., Nahmias, 1989). As I already stated, I was taught this in the early 1970s; I have also taught it this way many times since the late 1970s. Two sources that just preceded CC are also of interest. First is Morton and Pentico’s very comprehensive 1993 book about scheduling in general, which includes extensive coverage of project scheduling and multi-project scheduling. In the “MBC domain,” they describe OPT as an industrial benchmark, but also provide better sources for bottleneck-related scheduling. Their models for the CC environment precede CC and go way beyond what CC has to offer, both for single and multiple projects. Next, Raz and Marshall (1996) discuss the effects of resource constraints on project float (slack). It goes without saying that if you know the true slack, your critical path will not “jump all over” (Goldratt, 1997, Chapter 22, p. 212)-and in the case of Goldratt’s book, Chapter 22 of Critical Chain will become moot. Actually, we don’t even need to know the true slack to prevent such jumping: just follow the resource-constrained original schedule without factoring in slack (but include “soft” precedence constraints between activities that share a resource, after deciding how to sequence them). As Raz and Marshall (1996) demonstrate, the most popular project software package, Microsoft Project, had the capacity to do that as a matter of course (they cite Version 3.0, but such software capabilities were no longer new in the 1990s). In other words, the state of the art, as reflected by popular software, definitely included this consideration. But it is Chapter 22 of Goldratt’s book where the term “Critical Chain” is invented to respond to the “jumping critical path” phenomenon.

Interestingly enough, there is no need to look for earlier sources to prove the point. Goldratt himself, in a preemptive bid, provides sufficient evidence. To wit, consider the following quotes from Chapter 22 (Goldratt’s hero, Rick, a professor teaching a project management course, is narrating):

There are many topics I’ve skipped, but for a very good reason. They are what some would call academic-resource optimization, sequence optimization, investment optimization. I call them irrelevant, (p. 208, emphasis added)

And a bit later:

“Exactly,” I say. “Dependencies between steps can be a result of a path or a result of a common resource. Why are we so surprised that both dependencies are involved in determining the longest chain of dependent steps?” (p. 215)

Indeed, nobody should have been surprised. But, unfortunately, Rick had decided not to teach some “academic” and “irrelevant” topics, and thus deprived his students of the opportunity to draw the [trivial) practical and relevant conclusions. Nor does he hide this truth from his students:

“I don’t know how many articles dealing with resource sequence optimization I have read,” I tell them. “More than I care to remember. They contain many algorithms and heuristic rules to sequence resources. [. . .] But I don’t waste my time reading them anymore. Why? Because in each case the impact on the lead time of the project is less than even half the project buffer.” (p. 217)

Although it is not clear that the lead-time impact is indeed negligible, Goldratt is not the only one who believes that some theoretical scheduling models are impractical. But by including all sequencing rules in the group of redundant theory (e.g., all heuristics), he implied that the whole issue is not important. Yet, the need to sequence activities on limited resources is at the core of the critical sequence analysis!

The Abolishment of Due Dates

The second point, abolishing due dates, is perceptive and important. Nonetheless, it is not new either. Papers by Schonberger (1981) and Gutierrez and Kouvelis (1991) discussed this as an instance of Parkinson’s Law (i.e., work expands to fill the time allotted to it), and did so in the context of project management. Another source is W. Edwards Deming’s 14 Points (Deming, 1986), although Deming’s points are generic, and not specifically associated with the project management area. Deming had preached against the use of quotas and Management by Objectives (MBO) years before the advent of MBC, let alone CC. Managing by due dates is an instance of MBO, and, equivalently, a due date is a quota.

The Source of the Buffers Idea in the Academic Literature

Feeding buffers constitute one of the main innovations in CC relative to conventional CPM implementation. In general, a good project buffer is created when the stakeholders negotiate a reasonable and realistic completion date target. For the project manager, this allows minimizing the project cost, including the expected project delay penalties (e.g., penalties payable to the customer or other delay-related expenses). For the customers, it helps avoid the damage associated with overly optimistic expectations, and if delay penalties are negotiated, then customers are compensated for excessive delays (Ronen & Trietsch, 1988). Goldratt’s idea to monitor projects based on buffer penetration is not bad, although it requires corrections, but it is not the main function of the project buffer. Similar monitoring could easily be performed without an explicit project buffer. That is, instead of “buffer penetration,” we would monitor schedule slippage. (According to Goldratt’s approach, the size of the project buffer is arbitrary, so his fractional penetration measure is arbitrary too.) The use of a project buffer for its important protection purposes, however, is well known. One relatively early example is discussed by O’Brien (1965), under the name contingency (p. 142). In contrast, feeding buffers are important because they resolve the question when to start non-critical paths. In general, neither the early start policy nor the late start policy provides a correct approach to this question. Finally, when a project is really a subproject that feeds the critical path of a parent project, the project buffer is a feeding buffer of the parent project. One can say that feeding buffers are the general case and project buffers are a special case. Even when the project is not a subproject formally, it always serves some need that can be conceived as a parent project. Therefore, the project buffer is mathematically equivalent to a feeding buffer.

Feeding buffers may be important, but did Goldratt invent them? Ronen and Trietsch (1988) discuss a strongly related problem, and I can personally testify that it was clear to the authors that both project and feeding buffers should be incorporated in project planning. The focus of that paper is the determination of optimal feeding buffers for purchasing project items, but it also discusses project buffers and how to optimize them or negotiate them with customers. (There were two practical reasons for the limitation to this special case: a client needed it, and it is much more tractable than the general problem.) Kumar (1989), and Chu, Proth and Xie (1993) published essentially identical results for feeding buffers independently, thus demonstrating that the basic idea was understood by the professional community long before the publication of Critical Chain.

A direct conclusion of such models is that the time buffers of the various feeding activities should be balanced against each other, while taking into account their respective marginal costs and their propensity to limit the system, which we refer to as criticality (Elmaghraby, 1977, p. 27\7). This can be generalized for other buffers (e.g., resource capacity buffers should be balanced, too). Such balance may be viewed as an inescapable consequence of repeatedly implementing MBC’s Step 4 without waste, so one might expect Goldratt to applaud it. However, Goldratt is vehemently opposed to balance! His case against balance is argued in pages 139- 141 of Critical Chain, (concluding with the acronym “QED”), but the proof is based on a flawed assumption amounting to a tautology. Essentially, his argument is that if we wish to keep the bottleneck busy 100% of the time, we must have excessive capacity on other resources or the bottleneck will idle sometimes. The flaw is in the assumption that we have to keep the bottleneck busy at all times. Trietsch (in press-b) shows that correct balance involves matching criticalities with marginal resource costs. When applied to non- bottleneck resources in a system that allocates 100% of the criticality to a single bottleneck, the model shows marginal waste approaching 100%! Thus, this is a very serious mistake that Goldratt has made. Spearman (1997) provides another window into Goldratt’s surprising stance against balance, and the fact that people often accept it as true.

Finally, to conclude the discussion about buffers, there is an analogy between project buffers and bottleneck buffers, and between feeding buffers and assembly buffers. But there is also a major conceptual difference between them in terms of planning. As we have seen in the MBC arena, it is not necessary to plan individual buffers because CAPWIP (or other JIT implementations) will create them automatically. Instead, the relevant question is how much WIP we need in the factory to balance lead-time and throughput objectives. And we can answer the question by trial and error. In contrast, trial and error is not an option in project scheduling: all feeding buffers must be planned (which is why they are important). So, ironically, Goldratt’s contribution to buffers was superfluous in MBC and important (but not original) in CC. However, Goldratt also recommends a bottleneck buffer of project tasks in front of highly loaded multi-project [shared] resources: This, again, is superfluous. The buffers will create themselves. All that is required is to take the necessary additional queueing time into account. However, it is usually not necessary to schedule such resources very carefully in advance. Arguably, Goldratt’s own stance about the futility of optimization applies to this case.

Avoiding Multi-Tasking

It is easy to demonstrate that multi-tasking can be harmful. For example, in Portougal’s 1972 academic paper published in the Soviet Union, he showed that multi-tasking is detrimental in terms of all regular performance measures, for any project configuration. He assumed deterministic processing times, where task durations are inversely proportional to the intensity of resources allocated to them, and where all resources are interchangeable. He showed that there exists an optimal solution, where each job, once started, should be processed as quickly as possible. That is, we should never re-allocate resources in a manner that will delay the completion time of a task that had been started already.

Nonetheless, in practice, the idea should be taken with a grain of salt. First, while an optimal schedule exists that does not require preemption, this does not mean that preemption may not be required to correct mistakes (i.e., for control). Second, projects are never deterministic and resources are never completely interchangeable. For example, rework can never be scheduled in advance by a deterministic model. Or, suppose a manager is in the middle of a very long task, and three projects are waiting for preliminary approval of their plan. It is likely a good idea to serve them immediately and then return to the long job. (It is difficult to conceive of a manager who can do his/her job properly without multi-tasking.)

Similarly, CPU time-sharing, by completing very short jobs very quickly, was quite useful when it was first introduced. The explanation is that many programs were still at the debugging stage where it took the mainframe fractions of a second to diagnose them and, thus, provide the programmer with enough feedback to continue working. Yet, time-sharing is a quintessential example of multi- tasking. Goldratt’s philosophy is that if the resource is “the bottleneck,” then wasting capacity of the other resources is immaterial. But Trietsch (in press-b) shows that this philosophy is very wasteful for the system as a whole. It is wasteful in general, and not just in this instance. In conclusion, the idea is useful sometimes, but much less often than the hype around it seems to suggest.

Back to the USSR-The Source of the Bottleneck Focus Idea

While the main subject of this section is on the points in which CC looks different from CPM, it should be noted that the credit for Goldratt’s earlier ideas (e.g., the focusing idea incorporated within MBC) is also misdirected. One reason is that MBC, including the focusing aspect, is isomorphic to CPM. But it turns out that the focusing idea had also been articulated in the manufacturing arena long before MBC, at least in one industrialized nation: the Soviet Union. There were two schools of thought about managing production there, one “soft” and one mathematical. The former had promoted the idea of focusing on bottlenecks for several decades, starting in the first half of the last century. Considering Goldratt’s disdain of mathematical methods (as documented in this section), one can safely say that this group had basically taught the essential ideas Goldratt promoted with the same flavor, and did so before Goldratt was even born. The latter group picked up on the idea and started publishing mathematical papers about it long before the advent of OPT. For example, Pervozvansky (1975) wrote an extensive book on mathematical models in production management and discussed this idea in Chapter 7. The book also mentions an analytic solution of a flow shop with a distinct bottleneck, and discusses, in particular, the connections between resource constraints and the true critical path in projects! In other words, the ideas were known in the USSR both with respect to the MBC environment and the project environment, and they had been known years before Pervozvansky published his book- it’s just one source that certainly precedes Goldratt’s work. (My source of the information in this sub-section is my colleague Victor Portougal, an ex-Soviet scheduling expert, and I must say it came as a surprise to me when I first heard about it while working on this paper. Victor belonged to the mathematical school there, and the reference he provided is to one of the books in his personal library. As it happens, when he left the USSR, he only took with him relatively modern books. Thus, there is no reason to assume that Pervozvansky is an especially early reference for this idea, even within the mathematical school.)

5. On the Practical Pre-Critical Chain State of the Art

As we’ve seen, there is little in CC that was not known before, and some of it, including the excuse for the new name, was very well known. Does that mean that Goldratt contributed nothing new? The answer is a qualified “no.” In my opinion, Goldratt (or some unnamed associates of his) made an important contribution: He provided a new package. In it, he packed together items that go well together, but were marketed separately. Thus, he provided a more holistic approach than was previously the typical case. (A commercial law analogy comes to mind. The first person that attached erasers to pencils created a very convenient writing tool and can safely be called bright and innovative. Nonetheless, he merely combined well-known parts, and such combinations are not patentable. The inventor did not receive credit either for pencils or for erasers, and earned no royalties for the combination.) In effect, Goldratt provided a “one- stop shop” for these ideas, but he also ripped off all the labels from the merchandise before putting it on the shelves (not to protect the original brands, but, rather, to effectively re-brand them). This may be convenient for shoppers, but a far cry from a completely new approach and-from an academic point of view-there is no justification for hiding the sources of the items. 1 low, then, did Goldratt convince so many that it’s much more than merely a new package that he has to offer? Quite simply, instead of making claims with respect to CPM theory, he made [implicit] claims with respect to CPM as practiced by many and as presented by some books. Thus, he implied that typical CPM practitioners and books (1) do not identify the critical path correctly (by ignoring resource constraints), (2) fail to utilize and encourage earliness (by using due dates to manage individual activities), and (3) use the wrong approach to subordinate other activities to the needs of the critical path (which is where the feeding buffers come in).

Critical Chain’s hero, Rick, is assigned to teach project management for the first time in his career. He does not find the answers he needs in the textbooks the library carries. His disdain for academic papers has been mentioned before, so obviously he cannot use any of those. Therefore, together with his enthusiastic executive students (who liberally provide questions; answers; complete, successful, and instantaneous field-testing; and, last but not least, consulting opportunities) and with some support from colleagues, Rick proceeds to invent a “new” way to manage projects all with-in a one-semester course. Thus, the book astutely does not claim to improve upon the collected wisdom found in academic papers and advanced books, but rather, is pitted against some project management textbooks of the type available to the hero. Arguably, in a field th\at had been established about 40 years before the publication of Critical Chain, the viable textbooks should have reflected accumulated knowledge in the field, with the potential exclusion of esoteric material and very new contributions. But was this really the case?

Unfortunately, the answer is not entirely affirmative. Recently, I found myself in a similar situation, having to teach a full course in project management for the first time in my career. I was not a complete novice in the subject. I had taught CPM as a minor subject in POM and OR courses for many years, had worked within projects and project organizations, and had co-authored a couple of papers in the field. For a while, I toyed with the idea of teaching without a book altogether, but since I am not an expert in the general management aspects of the subject, I eventually decided to use a text. As a result of this experience, while I did learn many new things about the general management aspects, I also realized that the lessons I have learned and taught so often about CPM are not clearly presented in typical books.

Typical textbooks on the subject mention, as a matter of course, that resource leveling may change the length of the project, but they do not say clearly that the critical path is not fully identified until after all resource conflicts have been resolved. A Guide to the Project Management Body of Knowledge (Project Management Institute, 1996, Ch. 6)-which codifies one official collection of knowledge in the field-indicates that the critical path is not determined until after sequencing is done and, thus, contradicts Goldratt’s implicit claim (Globerson, 2000). Unfortunately, its coverage of this issue is quite confusing, and Rick could be justified in ignoring it for the purpose of teaching his course. The correct message is better conveyed in OR and POM books (e.g., Nahmias, 1989), but project management texts treat scheduling as a relatively minor issue.

A vicious cycle may be operating here: Scheduling methods, used incorrectly, did not work spectacularly well. In response, teachers (except those whose expertise is specifically in quantitative methods), and some books, stopped stressing them. Now they really don’t work well. Perhaps the two most important reasons for this are management by due dates and not using feeding buffers. (Sequencing is important, but there is abundant evidence that it was incorporated into practice already, although not necessarily optimally. Once we take sequencing into account somehow, there should be no reason to be unhappy with the results. After all, even if we miss the optimal solution by a lot, we will not know it! So this is not a likely cause of discontent. Instead, discontent is the result of failure to meet plans, and it is this failure that may have given project scheduling a bad reputation.)

Thus, overall, it seems that Goldratt successfully put his finger on an issue that OR teachers failed to market-as he had done before with respect to MBC. Somehow, important parts of the knowledge (and the intuition) that OR researchers and practitioners had developed- and published in academic and other outlets-failed to find its way to the textbooks we use to teach project management. Ironically, several recent textbook editions mention CC as the “source” of knowledge that sequencing may be required to remove resource contentions! This misses the boat in two important ways: First, as cited before, even Goldratt practically admits he barged through an open door there. So, why give him credit for it? Second, it misses two important items in the CC package: The avoidance of management by due dates and the specification of buffers. As we saw, these are not original Goldratt ideas either, but they are the ones where a strong argument could be made that practice does not conform to the recommendation, and the recommendation is meritorious.

6. Towards a More Holistic Approach

In general, CC provides a more holistic approach to project scheduling than the prevalent previous practice, and addressing the issues as a package is Goldratt’s true contribution, for which he and/or his associates deserve credit. We need to build on this basis and take a much more holistic approach to project management in general and to project scheduling in particular. Examples of a more holistic approach include (1) addressing existing techniques in the correct sequence, such as resolving resource contentions right after the determination of the initial critical path; (2) combining resource sequencing and scheduling with stochastic analysis; (3) combining crashing techniques with stochastic analysis (i.e., how to crash stochastic activities); (4) setting buffers optimally together with such scheduling and crashing activities; (5) planning buffers for time, capacity, cost, and scope in a concerted way by practical, theory-based methods; (6) monitoring buffers correctly (with a proper statistical quality control approach). Most of these go way beyond what CC has to offer, but the renewed debate CC caused may help bring this about. In this section, we discuss some of these issues.

What Should be Done Right Now?

The success of CC demonstrates that we can achieve immediate benefit by some relatively simple steps. We should not adopt the CC recommendations without scrutiny, however, since they include errors. Goldratt’s generic answer to such criticism is “it works” (see Cabanis-Brewin, 1999). But even if we stipulate that it does work, this just means that the errors are not fatal, not that they should be sanctioned. The fact is that Goldratt’s work requires both minor and major corrections. The minor corrections are easy to implement with the tools we currently have and, therefore, we should begin pursuing them at once. (From an academic point of view, this means we should start teaching them right now.) A reasonable sequence for scheduling projects that builds on the CC approach using existing tools would include the following seven steps:

1. Estimate durations (by deterministic or stochastic measures and without padding) and identify the absolutely essential precedence constraints (i.e., do not include any precedence constraint designed as an implicit resource constraint resolution).

2. Devise the CPM network and identify the initial critical path.

3. Identify any resource constraint violations and resolve them by judicious sequencing rules and/or software. (To prevent problems later, introduce “soft” precedence relationships into the network to enforce the sequencing decisions.)

4. Once a good, feasible plan has been devised, consider crashing. (Crashing before sequencing may crash activities that are not really critical, so crashing must follow sequencing, but the two can then be performed cyclically with each influencing the other.)

5. Set feeding buffers and a project buffer. Emphatically, if a feeding buffer is too small for comfort, do not change the sequence, and do not start a non-critical path before the critical path; instead, start both (or all) as early as possible and increase the project buffer as necessary. (Boldly ignore any contrary recommendations from various CC authorities. To delay the most critical path just because a nearly critical path does not have a large enough feeding buffer implies losing time with certainty to avoid any risk of losing the same time or less.)

6. Monitor performance based on buffer consumption. (However, Goldratt’s approach here fails to take into account basic statistical process control lessons and, therefore, this part can benefit from further research.)

7. In the old spirit of CPM and MBC (but contrary to Goldratt’s recommendation in CC), update the network (return to Step 1) whenever the critical path changes, or if buffer consumption is much more or less than expected. Before doing so, release all soft precedent constraints. (However, when the stability of the plan is very important, it is possible to treat some of these soft constraints as hard ones.)

Potential Future Work

Of course, this is just a start, based on what is easy to achieve relatively quickly. The list does not yet include nontime buffers and their coordination. It also does not specify how to crash stochastic activities and exactly how to set buffers (for a partial answer, see Trietsch, 2004).

Another open question is how to monitor buffers without tampering, based on statistical quality control principles balancing the damage from “Type I and Type II errors.” Coldratt’s recommendations for buffer setting and monitoring are better than nothing, but far from satisfactory. The correct estimation of project and activity durations based on historical data, and taking into account consistent bias (which varies across projects and introduces dependence between task duration estimates), is also important in this context. Incidentally, the existence of consistent bias implies that project buffers must be much larger than the randomness of individual activities suggests. This supports Goldratt’s heuristic of setting the buffer as a fixed proportion of the chain length when compared to the alternative of assuming independent activities. Leach (2000) includes an improvement whereby the buffer of short chains is relatively larger. Trietsch (in pressa) shows that the correct buffer is approximately given by a positive constant plus a fraction of the project duration. However, both the constant and the fraction should be based on historical data analysis and on the ratio between the cost of earliness and the project delay penalty. In contrast, Goldratt ignores the positive constant and uses an arbitrary fraction that is based neither on data nor on relative delay costs.

In general, Steps 3 to 7 highlight areas where more research is required. We have enough basic results to use now, but we also need more advanced results, since our present ones were derived by the analytic approach and are notfully satisfactory for the system as a whole. Morton and Pentico (1993) and Demeulemeester and Herroelen (2002) provide an excellent start for Step 3, and see also Trietsch (2004), but much more needs to be done. Another objective is to include resource capacity level optimization in the picture. This can fit within the cyclical application of Steps 3 and 4.

7. Conclusion

Not for the first time, Goldratt’s work led to debate and rethinking (e.g., Herroelen & Leus 2001-where many more such references may be found; Maylor, 2001; Raz et al., 2003). Such cause for thought is a good thing. However, while his influence cannot be denied and some of his teaching is brilliant, we should remember that Goldratt is not an academic, but an entrepreneur (albeit a bright and highly educated one). As such, his motivation may be different from that of academics. There is no compelling business reason for Goldratt (1) to give credit where it is due, (2) to adhere to the “academic” truth, (3) to discuss issues deeply and thoroughly, and (4) to make sure that his work can pass the scientific review hurdle that academic publications must. All this is fine as far as business is concerned. However, these issues are important to consider when we cite and evaluate Goldratt’s work as a source of academic knowledge. A critical approach is mandatory here.

Since practically all of Goldratt’s work had not been refereed, every academic who cites him should first imagine that he/she is required to evaluate Goldratt’s work, as would a referee. As for those who may feel unqualified to referee Goldratt’s work, they should be even more wary. The wide citation of Goldratt’s work in academic papers is not equivalent to a single thorough review of the type that all serious academic papers should undergo. (During my teaching career, I have seen the then-new edition of one of the most popular books in POM quote an erroneous example that Goldratt had taught one of the authors-a freshly minted Goldratt Associate. A simple LP check would have prevented that error, but Goldratt scorns LP. Evidently, the authors did not check Goldratt’s work with care. So, perhaps he could have cleared the peer review hurdle after all. Be that as it may, I tried to provide some of the “flavor” of such a review in this paper. After all, it is just one in a long series of papers written by academics who cite Goldratt.)

To counterbalance the criticism, however, we must note that CC- although identical to the old spirit of PERT/CPM-provides a fresh opportunity for improvement by a renewed holistic approach to project planning and execution. The paper lists some steps that can be taken almost immediately-such as changing the sequence in which we teach project scheduling models. It also discusses potential future enhancements based on studying project-scheduling issues in relevant combinations, rather than in isolation. Both practitioners and academics have a role in making this happen.

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DAN TRIETSCH, ISOM Department, University of Auckland, Old Choral Hall, 7 Symonds Street, Auckland

DAN TRIETSCH is Associate Professor of Operations Management at Auckland University. Previously, he taught at Tel Aviv University, Northwestern (Kellogg), Naval Postgraduate School (NPGS), and (as Professor of IE) at American University of Armenia. He holds a BSME from the Technion-Israel Institute of Technology, MBA (Cum Laude; specialization in OR) and PhD (Summa Cum Laude; specialization in transportation) from Tel Aviv University. Before his postgraduate studies, he had worked as an irrigation engineer on large projects. As a faculty member at NPGS, he acted as an internal consultant at several repair depots in the US Navy (large project organizations), combining IE and TQ methods with MBC to achieve focused improvements. Later, he was involved in industrial improvement projects in Armenia. His main publications appeared in Transportation Science, Transportation Research, SIAM Journal of Applied Mathematics, NETWORKS, Operations Research, European Journal of Operational Research, journal of the Society of Operational Research, IIE Transactions, Journal of Quality Technology, and Quality Engineering. He is also the author of a book on statistical quality control (published by World Scientific).

Copyright Project Management Institute Mar 2005

Fresh-Frozen Osteochondral Allograft Reconstruction of a Severely Fractured Talus: a Case Report

Talar fractures constitute

The use of autologous bone-tissue allografts (from the iliac crest or femoral head) to treat talar neck and body fractures has been reported. However, these allograft procedures have been accompanied by primary fusion of one or more of the seven articular surfaces of the talus, most commonly the talocalcaneal, talonavicular, and talotibial joints, leading to considerable loss of function2-4. The use of whole-bone fresh-frozen allografts in the foot has only been reported for the treatment of bone tumors. Muscolo et al. reported on the use of whole fresh-frozen calcaneal allografts for the treatment of chondrosarcoma and giant-cell tumor of the calcaneus5. We are unaware of any previous reports on the use of bulk allografts as an alternative to subtalar arthrodesis for the treatment of severely comminuted fractures of the talus. In an attempt to minimize loss of function, we reconstructed a severely comminuted talar fracture with use of a portion of a fresh-frozen osteochondral talar allograft. We report our results after twenty- nine months of follow-up. The patient was informed that data concerning this case would be submitted for publication.

Fig. 1

Preoperative lateral radiograph of the ankle, showing a comminuted fracture of the posterior part of the body of the talus extending into the subtalar joint.

Case Report

A fifty-year-old man fell from a height of 16 ft (4.9 m). He complained of pain in the back and in the right ankle. Radiographs demonstrated a comminuted fracture of the posteroinferior aspect of the right talus. The fracture involved the posterior aspect of the talar dome and extended anteroinferiorly, with severe comminution of the articular surface of the subtalar joint (Figs. 1 and 2), consistent with an Orthopaedic Trauma Association type 72-C2 fracture. Associated injuries included T12 and L1 spinal compression fractures. Because of the small size of the fragments and the extensive articular cartilage damage, the decision was made to reconstruct the talus by replacing the comminuted area of its posterior half and the subtalar articular surface with a fresh- frozen osteoarticular allograft.

Fig. 2

Axial computerized tomographic scan showing a severely comminuted posteroinferior fracture of the body of the talus. Note the small size of many of the fragments.

Fig. 3

Photograph of the anteromedial incision, showing the void created after excision of the comminuted fragments of the posterior half of the talar dome, including the totality of the subtalar articular surface.

A height, side, and gender-matched fresh-frozen talar allograft was ordered from the American Red Cross following tissue-bank guidelines. The surgery took place six days after the initial trauma, once the swelling had subsided and the allograft had become available. A 5-cm vertical posteromedial skin incision was performed between the medial malleolus and the Achilles tendon, with the incision curving around the medial malleolus toward the talonavicular joint. Next, a transverse osteotomy of the medial malleolus was performed to allow distal reflection of the medial malleolus and the deltoid ligament. A femoral distractor was placed across the ankle joint to facilitate exploration of the comminuted talus. The talus was exposed, and many severely comminuted fragments constituting the posterior half of the talar dome as well as the totality of the subtalar articular surface were excised, leaving a large posteroinferior void (Fig. 3). The posterior portion of the osteoarticular allograft was then fashioned to match the remaining intact anterior portion of the talus, and an anatomic reduction was obtained between the graft and the host bone (Figs. 4-A and 4-B). The allograft was secured to the remaining portion of the talus with two 4-0 partially threaded cancellous titanium screws that were placed, one from anterior to posterior and one from posterior to anterior, under fluoroscopic guidance. The osteotomized medial malleolus was then fixed with two lag screws.

Fig. 4-A

Photographs showing the contouring of the talar allograft (Fig. 4- A) and its insertion into the ankle joint (Fig. 4-B).

Fig. 4-B

The patient wore a posterior splint for ten days after surgery. Upon removal of the sutures, he was managed with a brace and was kept from bearing weight for ten weeks. The brace was removed three times a day for active and passive range-of-motion exercises. The patient started partial weight-bearing up to 40 lb (18.1 kg) at ten weeks and was advanced toward full weight-bearing at twelve weeks.

Fig. 5-A

Fig. 5-B

Anteroposterior (Fig. 5-A) and lateral (Fig. 5-B) radiographs of the ankle, made after twenty-nine months of follow-up, showing healing of the allograft to the retained portion of the native talus.

The patient was evaluated at two, four, six, twelve, twenty- four, and twenty-nine months. No complications were noted. At twenty- nine months, the range of motion of the ankle was slightly decreased, with 10 of dorsiflexion and 30 of plantar flexion (a 5 and 10 decrease, respectively, as compared with the contralateral ankle). The range of motion of the subtalar joint was measured with the patient lying prone. The hip was flexed 90, and the ankle was kept in neutral alignment at 90. Inversion and eversion stresses were applied to the foot, and the measurements were made with a goniometer. The subtalar range of motion was 5 of eversion and 10 of inversion. No limb-length discrepancy was observed, and no pain or loss of sensation was reported. The Musculoskeletal Function Assessment score6 was 19.32 points (possible range, 0 to 100 points, with lower scores indicating better function). Radiographs revealed intact hardware and no signs of osteonecrosis or nonunion (Figs. 5- A and 5-B). A magnetic resonance image of the foot, acquired after twenty-nine months of follow-up, revealed revascularization of the allograft (Fig. 6). At the time of the latest follow-up, the patient was able to use regular shoes and had returned to part-time work as a construction worker.

Fig. 6

T1-weighted sagittal magnetic resonance image of the talus, acquired after twenty-nine months of follow-up. Despite some artifact from the titanium screws, the grafted portion of the talus has a homogeneous vital signal.

Discussion

Talar fractures, although uncommon, often are associated with a poor prognosis and severe complications such as osteonecrosis, loss of range of motion, posttraumatic arthritis, and rapid joint deterioration1,7-10. Displaced fractures of the talar neck and body are best treated with open reduction and internal fixation2,3,8,10. As severely comminuted talar fractures have a very poor prognosis, arthrodesis may be necessary. Notably, Blair fusion7,8,11 and bone block distraction arthrodesis4 are intended to minimize the heel- height discrepancy that results from ankle fusion1.

Various authors have reported different morbidity rates following joint arthrodesis, perhaps because of differences in the initial severity or treatment of the fracture as well as in the method of arthrodesis2-4,8-11. Arthrodesis can result in nonunion or malunion of the joint, varus or valgus deformity, pain at the site of hardware, arthritis of adjacent joints, and reflex sympathetic dystrophy8,10,12.

The subtalar joint is a critical structure that affects the ability to absorb axial compression forces and to accommodate to uneven surfaces when walking. Proper function of the subtalar joint directly correlates with quality of life, and therefore the preservation of this function should be a priority. In the case of our patient, we reconstructed the posteroinferior aspect of the talus with a fresh-frozen osteoarticular allograft in an effort to restore maximum functional integrity after a severely comminuted fracture of the talus. At twenty-nine months of follow-up, the need for a tibiocalcaneal arthrodesis had been avoided and the patient had an excellent intermediate-term result. We recognize that our patient was extremely cooperative and motivated and that, without his help, the final outcome could have been different. Although the outcome after twenty-nine months of follow-up was very encouraging, longer follow-up is necessary in order to fully assess the advantages of this procedure compared with more standard treatments.

NOTE: The authors thank Donna Cece, PA, for her contributions to this work.

References

1. Higgins TF, Baumgaertner MR. Diagnosis and treatment of fractures of the talus: a comprehensive review of the literature. Foot Ankle Int. 1999;20:595-605.

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3. Schulze W, Richter J, Russe O, Ingelfinger P, Muhr G. Surgical treatment of talus fractures: a retrospective study of 80 cases followed for 1-15 years. Acta Orthop Scand. 2002;73:344-51.

4. Trnka HJ, Easley ME, Lam PW, Anderson CD, \Schon LC, Myerson MS. Subtalar distraction bone block arthrodesis. J Bone Joint Surg Br. 2001;83:849-54.

5. Muscolo DL, Ayerza MA, Aponte-Tinao LA. Long-term results of allograft replacement after total calcanectomy. A report of two cases. J Bone Joint Surg Am. 2000;82:109-12.

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9. Fleuriau Chateau PB, Brokaw DS, Jelen BA, Scheid DK, Weber TG. Plate fixation of talar neck fractures: preliminary review of a new technique in twenty-three patients. J Orthop Trauma. 2002;16:213-9.

10. Vallier HA, Nork SE, Benirschke SK, Sangeorzan BJ. Surgical treatment of talar body fractures. J Bone Joint Surg Am. 2003;85:1716-24.

11. Van Bergeyk A, Stotler W, Beals T, Manoli A 2nd. Functional outcome after modified Blair tibiotalar arthrodesis for talar osteonecrosis. Foot Ankle Int. 2003;24:765-70.

12. Robinson JF, Murphy GA. Arthrodesis as salvage for calcaneal malunions. Foot Ankle Clin. 2002;7:107-20.

BY IVAN F. RUBEL, MD, AND ALEXANDRA CARRER, MS

Investigation performed at SUNY Downstate-Kings County Medical Center, Brooklyn, New York

Ivan F. Rubel, MD

Alexandra Carrer, MS

Department of Orthopaedic Surgery and Rehabilitation Medicine, State University of New York Downstate Medical Center, 450 Clarkson Avenue, Box 30, Brooklyn, NY 11203

The authors did not receive grants or outside funding in support of their research or preparation of this manuscript. They did not receive payments or other benefits or a commitment or agreement to provide such benefits from a commercial entity. No commercial entity paid or directed, or agreed to pay or direct, any benefits to any research fund, foundation, educational institution, or other charitable or nonprofit organization with which the authors are affiliated or associated.

doi:10.2106/JBJS.C.01671

Copyright Journal of Bone and Joint Surgery, Inc. Mar 2005