Medicine and Health Sciences Commons^™

Volume 32, Index, Canadian Medical Association

Canadian Journal of Surgery

The mission of CJS is to contribute to the effective continuing medical education of Canadian surgical specialists, using innovative techniques when feasible, and to provide surgeons with an effective vehicle for the dissemination of observations in the areas of clinical and basic science research.

Visit the journal website at http://canjsurg.ca/ for more.

Uhs-Com Alumni Association Newsletter: Vol.1, No.3, Uhs-Com Alumni Association

Alumni Association Newsletter

Contents: The Alumni Association: Surviving the Changes -- Carl D. Claxton, D.O., Appointed Dean -- Board of Trustees Report -- Alumni Association President Urges Support -- In Memoriam -- Alumnus Moved to Contribute; Encourages Others to Respond -- Hotline Numbers You Should Know -- Dr. Whitten Provides An Update On The Status of The College -- New Board Member Pleasantly Surprised -- UHS Alumni Receive Honors, Awards -- Other Ways to Contribute -- Donation Options.

Volume 32, Issue 6, Canadian Medical Association

Canadian Journal of Surgery

The mission of CJS is to contribute to the effective continuing medical education of Canadian surgical specialists, using innovative techniques when feasible, and to provide surgeons with an effective vehicle for the dissemination of observations in the areas of clinical and basic science research.

Visit the journal website at http://canjsurg.ca/ for more.

Correspondence: Unecom: Kirmes To Lenz 1989-10-19, William Kirmes D.O.

William Kirmes, D.O. Collection

A letter from Dr. Kirmes to Dr. Craig Lenz discussing the Admissions Committee kick off lunch October 6, 1989 along with minutes from the meeting.

Volume 32, Issue 5, Canadian Medical Association

Canadian Journal of Surgery

The mission of CJS is to contribute to the effective continuing medical education of Canadian surgical specialists, using innovative techniques when feasible, and to provide surgeons with an effective vehicle for the dissemination of observations in the areas of clinical and basic science research.

Visit the journal website at http://canjsurg.ca/ for more.

Vital Signs, September 1989, Boonshoft School Of Medicine

Vital Signs

A twelve page newsletter created by the Boonshoft School of Medicine to document the current affairs of the school. This issue includes a variety of feature articles, alumni profiles, class notes, and more.

The Third International Workshop On Multiple Endocrine Neoplasia Type 2 Syndromes, Friedhelm Raue, Charles E. Jackson

Henry Ford Hospital Medical Journal

No abstract provided.

Front Matter

Henry Ford Hospital Medical Journal

No abstract provided.

Multiple Endocrine Neoplasia Type 2 Syndromes: Nomenclature Recommendations From The Workshop Organizing Committee, Robert F. Gagel, Charles E. Jackson, Bruce A. J. Ponder, Friedhelm Raue, Nancy E. Simpson, Reinhard Ziegler

Henry Ford Hospital Medical Journal

No abstract provided.

Hereditary Medullary Thyroid Carcinoma: Genetic Analysis Of Three Related Syndromes, Hagay Sobol, Steven A. Narod, Isabelle Schuffenecker, Chris Amos, R. Alan B. Ezekowitz, Gilbert M. Lenoir

Henry Ford Hospital Medical Journal

Hereditary medullary thyroid carcinoma (MTC) appears in three forms: 1) in association with pheochromocytomas and parathyroid hyperplasia (multiple endocrine neoplasia type 2 A [MEN 2A]); 2) with pheochromocytomas, neuromas of the mucous membranes, and a marfanoid appearance (MEN 2B): and 3) without pheochromocytoma. Despite these differences in presentation, age of onset, and clinical severity, limited genetic studies suggest that the three MTC variants may be due to inherited mutations at the same gene locus. We present further data in support of the hypothesis that allelic variation may underlie the diversity of these endocrine neoplasia syndromes.

Closing In On The Men2a Locus, Nancy E. Simpson, Kenneth K. Kidd

Henry Ford Hospital Medical Journal

The mapping of the locus for multiple endocrine neoplasia type 2A (MEN 2A) to chromosome 10 using linkage is briefly reviewed including a discussion of linkage strategy and reference to some of the exclusions before the assignment. The subsequent development of the map of the centromeric region of the chromosome and the linking of what appear to be the four closest flanking markers and the centromeric alphoid sequence to the disease locus are reviewed. To date no recombination has been observed between the centromeric marker and the MEN2A locus among, at least, 26 informative meioses, 11 of which are phase …

Screening For Medullary Thyroid Cancer In France: A National Effort, Claude Calmettes, A. Chaventre, Nicole Feingold, Brigitte Franc, J. M. Guliana

Henry Ford Hospital Medical Journal

Screening for medullary thyroid cancer (MTC) in France is based on a protocol that has been widely distributed nationally. A network of coordinators utilizing a common questionnaire provides for an effective national screening program. Calcitonin stimulation procedures are systematically used for all first-degree relatives of MTC patients. Pathological studies utilize special immunopathologic techniques. Genealogic information is obtained on all index cases, and blood specimens are collected for establishing permanent cell lines. The data collected are used not only to establish the diagnosis of the hereditary or sporadic form of the disease but also to expand the screening as appropriate. This …

Allele Loss On Chromosome 10 And Point Mutation Of Ras Oncogenes Are Infrequent In Tumors Of Men 2 A, Makoto Okazaki, Akihiro Miya, Norifumi Tanaka, Tetsuro Miki, Masayuki Yamamoto, Kazuyoshi Motomura, Akira Miyauchi, Takesada Mori, Shin-Ichiro Takai

Henry Ford Hospital Medical Journal

The multiple endocrine neoplasia type 2A (MEN 2A) gene has been mapped to the centromeric region of chromosome 10 by linkage analysis. We examined 36 medullary thyroid carcinomas (MTCs) (16 hereditary and 20 sporadic) and ten pheochromocytomas (eight hereditary and two sporadic) to detect loss of alleles on chromosome 10 using seven polymorphic DNA markers mapped to this chromosome. Of 20 informative cases, only one (5%) sporadic MTC showed loss of heterozygosity at the locus RBP3. Allele loss at the RBP3 locus was not found in pheochromocytomas from six heterozy gates. All tumors retained constitutional heterozygosity at six other loci …

The Importance Of Screening For Medullary Thyroid Carcinoma In Families Of Patients With Men 2, A. Frilling, P. E. Goretzki, L. Bastian, H. D. Roeher

Henry Ford Hospital Medical Journal

Family .screening for medullary thyroid cancer (MTC) is important for detecting members of multiple endocrine neoplasia type 2 (MEN 2) families who may be gene carriers but show no clinical evidence of the disease. Most members of our MEN 2 families are screened yearly by measuring basal and pentagastrin-stimulated calcitonin (CT) levels. A 15-year-old first-degree relative of an affected member of the D-kindred showed a normal basal and an elevated stimulated CT level. Clinical examination, ultrasonography, and scintigraphy were normal. Thyroidectomy and bilateral neck dissection revealed a multicentric MTC with no lymph node involvement. In the O-kindred we detected elevated …

Men 2a: Update On The Northern Ireland And Australian Family, Patrick J. Morrison, David R. Hadden, Colin J. Russell, Norman C. Nevin

Henry Ford Hospital Medical Journal

The Northern Ireland/Australian family with multiple endocrine neoplasia type 2A (MEN 2A) originally described in 1987 is presented with a revised and enlarged pedigree. Four members of the first generation studied have died. A seventh member of the second generation studied has developed medullary thyroid carcinoma and has progressed to surgery. None of the third generation members studied has shown any conclusive abnormality in metabolic screening tests. Each member of the third and fourth generations has had genetic counseling and (if appropriate) DNA analysis with gene probes close to the MEN 2A gene locus on chromosome 10. All members of …

Results Of A Screening Program For Multiple Endocrine Neoplasia Type 2a: A Clinical Study Of A Japanese Family, Takashi Shimotake, Naomi Iwai, Jun Yanagihara, Tetsuro Kobayashi, Shin-Ichiro Sakai

Henry Ford Hospital Medical Journal

A Japanese family of 87 members in five generations with multiple endocrine neoplasia type 2A (MEN 2A) is described regarding the utility of screening tests for early detection of medullary thyroid carcinoma and the potential for DNA diagnosis of MEN 2A gene carriers. The screening programs for family members in this series include measurements of plasma calcitonin concentrations after intravenous injection of pentagastrin (0.5 μg/kg/5 sec) and 24-hour urinary excretion of catecholamines. While 18 MEN 2A patients had been previously diagnosed, these screening programs revealed five additional patients with MEN 2A (aged 16, 19, 35, 37, and 57). Prediction of …

Screening For Pheochromocytoma In The Men 2 Syndrome, J. Kotzerke, C. Stibane, H. Dralle, H. Wiese, W. Burchert

Henry Ford Hospital Medical Journal

Twenty-two patients with the multiple endocrine neoplasia type 2 (MEN 2) syndrome were screened for pheochromocytoma since it is a major cause of morbidity in MEN 2 families. Clinical symptoms, biochemical parameters, ultrasound, computed tomography or magnetic resonance imaging, and meta-iodo-benzylguanidine (MIBG) scintigraphy were evaluated for detection of adrenal tumors. Clinical symptoms and plasma or urine catecholamines appeared to be nonspecific, whereas MIBG scintigraphy was highly specific and the most sensitive parameter. Patients older than age 30 should be scintigraphically screened at least once despite the radiation exposure. Demonstration of only slight uptake is not an indication for surgery but …

Comparison Of Sporadic And Hereditary Forms Of Medullary Thyroid Carcinoma, M. Rosenberg-Bourgin, P. Gardet, R. De Sahb, M. Schlumberger, B. Caillou, M. Guilloud-Bataille, J. P. Travagli, N. Feingold, C. Parmentier

Henry Ford Hospital Medical Journal

Between 1960 and 1988, 185 patients with medullary thyroid carcinoma (MTC) were followed at the Institut Gustave Roussy in France. The screening of the family members by calcitonin measurement (basal or after pentagastrin stimulation) has led lo the characterization of 38 sporadic cases and 44 hereditary cases. Among the hereditary cases are seven families with MTC only and two families with multiple endocrine neoplasia type 2A (MEN 2A). MTC only cases and MEN 2B cases are present as apparently sporadic forms. Hereditary cases consisted of 26 females and 18 males; the male-female ratio was 21:17 in sporadic cases. Ten of …

Diagnosis, Management, And Pathogenetic Studies In Medullary Thyroid Carcinoma Syndrome, Naguib A. Samaan, Kuo-Pao Paul Yang, Pamela Schultz, R. C. Hickey

Henry Ford Hospital Medical Journal

A retrospective study of 224 patients with medullary thyroid carcinoma (MTC) diagnosed between 1963 and 1988 was performed to 1) establish the diagnosis of MTC in early childhood, 2) establish the role of prophylactic regional lymphadenectomy in patients with MTC, 3) study the effect of chemotherapy on MTC patients with metastatic disease, 4) study the effect of somatostatin analog 201-995 (Sandoz Pharmaceuticals) on the frequency of diarrhea in MTC, and 5) locate the common region(s) of gene deletion on chromosome 1 and examine the loss of heterozygosity on chromosome 10 in tumors. Our data indicated that a progressive rise of …

Postoperative Pentagastrin-Stimulated Serum Calcitonin Concentrations In Patients With Medullary Thyroid Carcinoma: Reoperations In Patients With Concentrations Bordering The Detection Limit, Hinrich Meybier, Heinrich Schmidt-Gayk, Heinz Buhr, Friedhelm Raue

Henry Ford Hospital Medical Journal

The case reports on two patients with medullary thyroid carcinoma show that even postoperatively stimulated serum calcitonin (CT) concentrations near the detection limit (using a polyclonal antibody against synthetic CT) can demonstrate persistent disease. Stimulated CT concentrations can be lowered to nondetectable levels by a second and third operation if a meticulous technique is used for dissection of the lymph compartments. The patient can then be assumed lo be cured. Diagnostic accuracy at very low CT concentrations can be improved by selective venous catheterization with blood sampling for CT after stimulation.

Cutaneous Lichen Amyloidosis Associated With Multiple Endocrine Neoplasia Type 2a, V. Nunziata, G. Di Giovanni, A. M. Lettera, M. D'Armiento, M. Mancini

Henry Ford Hospital Medical Journal

We have previously described a kindred with hereditary medullary thyroid carcinoma and pheochromocytoma (multiple endocrine neoplasia type 2 A [MEN 2A]) with localized pruritic cutaneous manifestations present only in affected members. Although the initial skin biopsies reported did not show amyloidosis, subsequent skin biopsy results reported here have demonstrated amyloid which stained for keratin but not for calcitonin and established that this family represents an association of a rare autosomal dominant form of lichen amyloidosis with MEN 2A.

Surgical Treatment Of Medullary Thyroid Carcinoma In A Thirteen-Year-Old Girl With Men 2b, Lars-Erik Tisell, Svante Jansson

Henry Ford Hospital Medical Journal

Patients with palpable medullary thyroid carcinoma (MTC) have lymph node metastases in 90% of cases. In most series such patients continue to have elevated serum calcitonin (CT) levels after surgery indicating residual tumor tissue. We attempted a microdissection technique for the treatment of MTC. ' 'Micro'' refers not to a small incision or a limited exploration but to a more safe operation associated with good lighting and magnification and minimal bleeding. This technique was used in a 13-year-old girl with multiple endocrine neoplasia type 2B (MEN 2B). The prognosis for MTC in MEN 2B is worse than for sporadic MTC …

Adrenal Cortex Transplantation After Bilateral Total Adrenalectomy In The Rat, Georg F. W. Scheumann, Wolfgang F. A. Hiller, Soren Schroeder, T. Schurmeyer, Jurgen Klempnauer, Henning Dralle

Henry Ford Hospital Medical Journal

An experimental animal model with adrenal cortex transplantation was developed to study adrenal cortex replacement therapy in patients with multiple endocrine neoplasia type 2 who have had bilateral adrenalectomy for pheochromocytomas. Adrenal cortex of syngenetic rats was isolated from the medulla by collagenase digestion and a defined sedimentation. The cell suspension of the cortical cells was implanted under the kidney capsule of untreated syngenetic rats. After two weeks the recipients were bilaterally adrenalectomized. Serum corticosterone levels were measured as an estimate of function of the grafts. All recipients were healthy throughout the observation period, whereas all adrenalectomized controls died within …

Familial Cutaneous Lichen Amyloidosis In Association With Multiple Endocrine Neoplasia Type 2a: A New Variant, D. T. Donovan, M. L. Levy, E. J. Furst, B. R. Alford, T. Wheeler, J. A. Tschen, R. F. Gagel

Henry Ford Hospital Medical Journal

Multiple endocrine neoplasia type 2A (MEN 2A) is a rare hereditary disease transmitted in families as an autosomal dominant trad. We have identified a family in which the expression of a rare autosomal dominant form of cutaneous lichen amyloidosis appears to cosegregate with MEN 2A. In this family the skin lesion presented as multiple infiltrated papules overlying well demarcated plaques over the scapular area (right or left). Immunohistochemical studies demonstrated amyloid which stained for keratin but not calcitonin. A total of 19 members were screened. Three members of the family have the characteristic skin lesion and MEN 2A; two additional …

Quality Of Life After Bilateral Adrenalectomy In Men 2, M. Telenius-Berg, M. A. Ponder, B. Berg, B. A. J. Ponder, S. Werner

Henry Ford Hospital Medical Journal

Pheochromocytoma is a major cause of morbidity and mortality in the multiple endocrine neoplasia type 2 (MEN 2) syndrome. For the physician, surgical treatment seems well justified even though bilateral adrenalectomy will induce iatrogenically complete loss of adrenocortical function. For the patient this treatment may be a cause of medical problems as well as worry. We have evaluated quality of life after bilateral adrenalectomy in 27 MEN 2 patients through a combined oral and written approach. Mortality was low (one of 27), as was serious morbidity. Most patients had adapted well to the postadrenalectomy state. However, fatigue, worry, and noncompliance …

Living With A Hereditary Form Of Cancer: Experiences And Needs Of Men 2 Patients And Their Families, Marc P. H. D. Cleiren, Wil Oskam, Cornelis J. M. Lips

Henry Ford Hospital Medical Journal

Unlike the purely medical research done in multiple endocrine neoplasia type 2 (MEN 2) families, little work has been done on the psychosocial aspects of the disease. To assess the severely stressful influences and the consequences of that stress on the family network, a small-scale survey was carried out during a national meeting. The goal of the study was to obtain more information about the experiences and needs of MEN 2 patients and their relatives. Of the 59 respondents, 85% were satisfied with the medical information provided, 81% were satisfied with the medical knowledge of the specialist, but only 12% …

Use Of Somatostatin Analog Sms 201-995 In Medullary Thyroid Carcinoma, Alfonso Libroia, Uberta Verga, Gianleone Di Sacco, Marco Piolini, Fabrizio Muratori

Henry Ford Hospital Medical Journal

We have studied seven subjects with medullary thyroid carcinoma. Each had elevated basal serum calcitonin (CT) levels following total thyroidectomy. After subcutaneous administration of 100 μg of SMS 201-995, blood samples were collected at 60-minute intervals for six hours. Two patients showed a marked decrease of CT levels (patient A: baseline 565 μg/mL, nadir 150 μg/mL; patient B: baseline 1,632 μg/mL, nadir 416 μg/mL). The other five patients showed no significant change in comparison with saline infusion. Two patients were treated with SMS 201-995 (300 μg/day)for 90 days. One of these patients responded to the acute SMS 201-995 test and …

Experience In Imaging Medullary Thyroid Carcinoma Using 99mtc (V) Dimercaptosuccinic Acid (Dmsa), Susan Clarke, Colin Lazarus, Michael Maisey

Henry Ford Hospital Medical Journal

99mTc (V) dimercaptosuccinic acid (DMSA) is a new tumor imaging agent that has been successfully used to image patients with medullary thyroid carcinoma (MTC). Since 1986, studies have been performed in 32 patients with histologically proven MTC at Guy's Hospital, London, England. Five patients with primary tumor were studied prior lo surgery, four patients were studied after successful removal of the primary tumor, and 26 patients with biochemical evidence of recurrence were studied. Eight patients were studied serially to assess progression of disease, and four patients were studied before and after surgery. Twenty-one of the 26 patients with disease had …

Contribution Of Different Scintigraphic Techniques To The Management Of Medullary Thyroid Carcinoma, Dirk Sandrock, Hans-Christian Blossey, Martina Steinroeder, Dieter L. Munz

Henry Ford Hospital Medical Journal

We compared three differenl scintigraphic techniques for the localization of neck recurrences and metastases in seven patients wiih medullary thyroid carcinoma one month to eight years after the first surgical intervention. Three successive scintigraphic studies were performed in five patients (6x3 studies) within two weeks using 201Tl chloride, 111In-labeled F(ab')2 fragments of the anticarcinoembryonic antigen (anti-CEA) monoclonal antibody (MoAb) BW 431/31. and 131I meta-iodobenzylguanidine (MIBG). Additionally, 11 studies were performed wilh the 111In-labeled MoAb fragment BW 431/31 (seven studies) or the 99mTc-labeled intact anti-CEA MoAb BW 431/26 (four studies). The gold standards for classifying scintigraphic results were biopsy, histology, surgery, …

The Role Of Radiopharmaceuticals Mibg And (V) Dmsa In The Diagnosis Of Medullary Thyroid Carcinoma, Uberta Verga, Fabrizio Muratori, Gianleone Di Sacco, Franceso Banfi, Alfonso Libroia

Henry Ford Hospital Medical Journal

The diagnostic value of 123/131I meta-iodo-benzylguanidine (MIBG) and 99mTc (V) dimercaptosuccinic acid (DMSA) was investigated in 12 patients with proven medullary thyroid carcinoma (MTC). Scintigraphic imaging with DMSA was negalive in nine of 12 patients. Scintigraphy with MIBG was positive in only one case. In proven primary or recurrent disease, DMSA sensitivity was 50% and MIBG sensitivity was 25%. Such sensitivities become much lower in subjects wilh high calcitonin (CT) levels who have had negative surgical explorations: DMSA 17% and MIBG 0%. DMSA delected tumor in 25% of the patients and MIBG in only 8%. The positivity of these scintigraphies …