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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 …

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.

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 …

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.

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 …

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 …

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 …

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 …

Localization Of Metastases From Medullary Thyroid Carcinoma Using Different Methods, Rosa C. Cabezas, Luis Berna, Montserrat Estorch, Ignacio Carrio, Angeles Garcia-Ameijeiras

Henry Ford Hospital Medical Journal

We analyzed the efficiency of three different noninvasive methods in the localization of recurrent medullary thyroid carcinoma (MTC). Nine patients (six females and three males) with biochemical evidence of disease after primary surgery were subjected to anti-carcinoembryonic antigen (anti-CEA) antibody, meta-iodo-benzylguanidine (MIBG), and computed tomography. Another female patient, in biochemical remission for six years after initial surgery, was also studied using the same methods. Three of the ten patients had negative results with all three methods (including the patient in remission). The other seven patients showed abnormal uptake of labeled anti-CEA antibody in various localizations: only two of these patients …

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 …

Diagnostic And Therapeutic Potential Of New Radiopharmaceutical Agents In Medullary Thyroid Carcinoma, Luigi Troncone, Vittoria Rufini, Giovina De Rosa, Amerigo Testa

Henry Ford Hospital Medical Journal

Recently developed radiopharmaceuticals have been proposed for imaging medullary thyroid carcinoma (MTC) with some having therapeutic potential. This study compares the imaging results obtained with radioiodinated meta-iodo-benzylguanidine (MIBG), 99mTc (V) DMSA, and 131I F(ab')2 anti-carcinoembryonic antigen (anti-CEA) in a group of MTC patients. In 23 patients 131I MIBG imaging showed a high specificity (no false-positive results) but a less satisfactory sensitivity (50%). In 12 patients 99mTc (V) DMSA revealed a better sensitivity (77%) but a lower specificity (three false-positive results). Positive results were obtained in two of three patients studied with 131I F(ab')2 anti-CEA. These data suggest that the highly …

Dna Cytophotometric Findings In Pheochromocytoma, Barbara-Christina Padberg, Esther Garbe, Eike Achilles, Henning Dralle, Max Bressel, Soren Schroeder

Henry Ford Hospital Medical Journal

Fifty adrenalectomy specimens containing normal (n = 3), hyperplastic (n =4), or neoplastic (n = 43) medullary tissue were subjected lo quantitative measurements of DNA content. Of the 43 pheochromocytomas, 16 were neoplasms inherited in the setting of multiple endocrine neoplasia type 2A. Five of 27 sporadic pheochromocytomas followed a malignant clinical course. Follow-up data were available in 25 patients. In normal medulla and adrenomedullary hyperplasia, either diploid or euploid DNA distributions were found. In contrast, 87% (33 of 38) of the benign and all five malignant pheochromocytomas exhibited nondiploid or aneuploid DNA histograms. No differences in DNA content existed …

A Preliminary Evaluation Of Calcitonin And Pdn-21 As Tumor Markers For Lung Cancer, J. J. Body, J. C. Dumon, J. P. Sculier, G. Dabouis, H. Lacroix, P. Libert, M. Richez, G. Bureau, P. Mommen, N. Raymakers, M. Paesmans, J. Klastersky

Henry Ford Hospital Medical Journal

Immunoreactive calcitonin (ICT) can be ectopically secreted by lung cancer cells and has been proposed as a tumor marker for bronchial neoplasms. Since PDN-21 (katacalcin or the carboxyl-terminal flanking peptide of the calcitonin gene) and CT are cosecreted in normal subjects and in patients with medullary thyroid carcinoma (MTC), we sought to determine the potential utility of PDN-21 as a tumor marker for lung cancer. We measured carcinoembryonic antigen (CEA), neuronspecific enolase (NSE), iCT, and PDN-21 in 119 to 378 healthy subjects, 88 to 91 patients with benign pulmonary diseases, and 249 patients with advanced lung cancer (108 small cell …

Modulation Of Calcitonin Secretion By Modification Of Calcium Channels?, Hans Scherubl, Friedhelm Raue, Michael Hoflich, Reinhard Ziegler

Henry Ford Hospital Medical Journal

Voltage-dependent calcium channels (VDCC) regulating Ca++ influx through the cellular plasma membrane play a major role in the Ca++ -induced calcitonin (CT) secretion. Using rat C-cells (rMTC 6-23 cell line), we have studied the effect of repetitive stimulation by either Ca++ (2 mM) or glucagon (10 μM) or epinephrine (10 μM) on CT secretion. Following a Ca++ -induced initial rise, CT release declined to basal levels after about four hours despite high Ca++ ; addition of 10 μM glucagon to the "Ca++ desensitized C-cells" yielded the normal stimulatory effect of glucagon on CT release. Repetitive stimulation with glucagon showed a …

Back Matter

Henry Ford Hospital Medical Journal

No abstract provided.

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.

Recurrent/Persistent Pneumonia In A 3 1/2-Year-Old-Girl Due To Acquired Immune Deficiency Syndrome., Ricardo Castro, Md, Joel Klein, Md, Rajeswary Padmalingam, Md, Stephen C. Eppes, Md

Department of Pediatrics Faculty Papers

The following is the report of the first case of a Delaware born child who developed AIDS, born from a mother who was not in a high risk camp.

Uhs-Com Alumni Association Newsletter: Vol.1, No.2, Uhs-Com Alumni Association

Alumni Association Newsletter (1989-1990)

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; Encourage Others to Respond -- Hotline Numbers You Should Know -- Dr. Whitten Provides An Update On The Status of The College -- For Those Born Before 1945 -- Other Ways to Contribute -- UHS Alumni Receive Honors, Awards -- A New Alumni Board Member Speaks -- SOS Donation Options.

The Concentration Of Prostaglandin E2 In Periradicular Lesions, Steve N. Mcnicholas

Loma Linda University Electronic Theses, Dissertations & Projects

Prostaglandins (PG) have been implicated in pathogenesis of periapical lesions. The purpose of this study was to determine the concentrations of PGE₂ in human periapical lesions. Periapical specimens from patients with clinical signs and symptoms of chronic and acute apical periodontitis were obtained and immediately frozen in liquid nitrogen. Periradicular tissues from unerupted third molars were frozen and used as controls. The concentration of PGE₂ was determined by radioimmunoassay. Low levels of PGE₂ were found in the control tissues as compared to those detected in chronic and acute lesions. A significantly higher level of PGE₂ was …

Transforming Growth Factor-Beta Stimulation Of Lung Fibroblast Prostaglandin E2 Production., Arturo Diaz, John Varga, Sergio A. Jimenez

Department of Medicine Faculty Papers

Transforming growth factor-beta (TGF beta) stimulated the production of total protein, collagen, and fibronectin by normal human lung fibroblasts. The stimulatory response was maximal at 100 pM TGF beta and reversed toward control at higher concentrations. Inhibition of fibroblast prostaglandin (PG) synthesis enhanced TGF beta-induced stimulation of total protein, collagen, and fibronectin production and reversed the negative slope of the dose-response curve at high concentrations of TGF beta. Determination of the steady-state levels of Types I and III procollagens and fibronectin mRNAs employing specific cDNA probes demonstrated that inhibition of fibroblast PG production increased the stimulatory effect of TGF beta …

Identification And Ranking Of Stressors In Nuclear Medicine Technology, Scott R. Sechrist

Community & Environmental Health Theses & Dissertations

The purpose of this study was to identify the most significant stressors in the field of nuclear medicine technology. A sample of convenience of 25 nuclear medicine technologists was used to generate a list of 35 stressors specific to the occupation. Sixty-three randomly selected nuclear medicine technologists responded to a mailed questionnaire which asked the technologist to assess the relative stressfulness of each of the 35 items on a scale of "0 to 1000". Data from the 59 valid responses indicated that equipment malfunctions, add on exams, uncooperative physicians, lack of staff, and uncooperative patients were the most significant stressors …

Digest Of The Philadelphia College Of Osteopathic Medicine (Summer 1989), Philadelphia College Of Osteopathic Medicine

Digest

The Summer 1989 issue of the Digest of the Philadelphia College of Osteopathic Medicine includes the following articles:

• President's Perspective
• Alumni in Practice
• Swinging on the Hinges of Fate
• Building a Better Thinking Cap
• The Recruiters Crystal Ball
• Seventy-Five Measurements per Second
• On Campus
• Class Acts
• In Memoriam
• Calendar

Maternal-Neonatal Serum Vitamin A Concentrations., Girish S. Shirali, D G. Oelberg, K P. Mehta

Manuscripts, Articles, Book Chapters and Other Papers

Prevention of neonatal vitamin A deficiency is related to the adequacy of maternal vitamin A stores. In this study we investigated maternal and cord serum vitamin A and retinol-binding protein (RBP) values in an Indian population including, for the first time, clinically vitamin A-deficient mothers. Twenty-eight maternal-neonatal pairs were selected from maternal cohorts of high socioeconomic status without clinical evidence of vitamin A deficiency (group I) and low socioeconomic status with conjunctival xerosis and Bitot's spots (group II). Maternal education, caloric and vitamin A intakes, weight, height, hemoglobin, and birth weight were significantly lower in group II. Serum vitamin A …

Microwell Based Competitive Enzyme Immunoassay For Quantifying Serum Triiodothyronine And Thyroxine Levels, Yiwen Yang

Chemistry & Biochemistry Theses & Dissertations

A microwell based competitive enzyme immunoassay for determination of total thyroxine and triiodothyronine in human serum was developed with use of horseradish peroxidase conjugate. The assay, which can be performed in only 1.5 hours at room temperature, involves simultaneous incubation of T₃- or T₄- HRPO conjugate and serum sample in anti-T₃ or anti-T₄ gamma globulin fraction coated polystyrene microwells.

Isolation of anti-T₃ and anti-T₄ gamma globulin fractions were accomplished from whole antiserum by using DEAE-cellulose chromatograph. A carbodiimide coupling method was described to prepare the T₄- and T₃-HRPO …

Department Of Radiology-Annual Report-July 1, 1988 To June 30, 1989, David C. Levin

Department of Radiology Administrative Documents and Reports

Department of Radiology Annual Report, July 1, 1988 to June 30, 1989. Thomas Jefferson University Hospital, Philadelphia, Pennsylvania, United States. 81 pages.

Robert S. Knighton, Md, Ghaus M. Malik

Henry Ford Hospital Medical Journal

No abstract provided.

Back Matter

Henry Ford Hospital Medical Journal

No abstract provided.

Recurrent Leukemia Cutis In Acute Myeloblastic Leukemia, Reda A. Girgis, Howard Terebelo, Koichi Maeda

Henry Ford Hospital Medical Journal

We report the case of a 64-year-old female with acute myeloblastic leukemia (French-American- British classification: M2) who developed two specific cutaneous manifestations during her illness. She presented with extensive cellulitis involving the face, neck, and upper chest wall. While the cellulitis resolved with antibiotic therapy, a fungating ulcerated nodule remained on the lower lip which proved to be leukemic on biopsy. Concomitant blood and bone marrow findings were diagnostic of acute myeloblastic leukemia. The lip lesion cleared with a course of chemotherapy. An erythematous macular rash subsequently developed over the lower trunk which was thought to be an allergic reaction …