Medical Specialties Commons^™

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.

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 …

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 …

Myasthenia Gravis, Stanton B. Elias

Henry Ford Hospital Medical Journal

No abstract provided.

Front Matter

Henry Ford Hospital Medical Journal

No abstract provided.

Hypoglycemia After Administration Of Somatostatin Analog (Sms 201-995) In Metastatic Carcinoid, John E. Brunner, Davida F. Kruger, Michael A. Basha, Eyal Meiri, Scott S. Kaatz

Henry Ford Hospital Medical Journal

SMS 201-995 (Sandoz Pharmaceuticals. East Hanover NJ) is a synthetic peptide analog of native somatostatin that has been used to relieve .symptoms caused by neuroendocrine tumors. Reports have described an insulin suppressive effect of SMS 201-995 that results in elevations of blood glucose. We report a patient with a metastatic small bowel carcinoid and renal failure in whom mild symptomatic hypoglycemia occurred 30 to 60 minutes after SMS 201-995 administration. No increase in insulin or decreases in glucagon. Cortisol, or catecholamines were observed during these hypoglycemic episodes. Elevated levels of growth hormone fell gradually following SMS 201-995 administration and did …

Clinical And Laboratory Features Of Small Left Atrial Myxomas, Gerardo A. Polanco, Mohsin Alam, Allan M. Haggar, Remigio Garcia

Henry Ford Hospital Medical Journal

No abstract provided.

A Suggested New Approach To Supraventricular Tachydysrhythmia After Coronary Bypass Surgery, Walter Kao, Mihai Gheorghiade

Henry Ford Hospital Medical Journal

No abstract provided.

Toxic Shock-Like Syndrome Associated With Necrotizing Streptococcus Pyogenes Infection, Thomas J. Connolly, Donald J. Pavelka, Eugene F. Lanspa, Thomas L. Connolly

Henry Ford Hospital Medical Journal

Two patients with toxic shock-like syndrome are presented. Both patients had necrotizing cellulitis due to Streptococcus pyogenes, and both patients required extensive surgical debridement. The association of Streptococcus pyogenes infection and toxic shock-like syndrome is discussed.

Ulceroglandular Tularemia: A Typical Case Of Relapse, Stanley D. Miller, Michael B. Snyder, Michael Kleerekoper, Colby H. Grossman

Henry Ford Hospital Medical Journal

Tularemia is an infectious disease that continues to occur sporadically and in epidemics in the United States. It is characterized as an acute febrile illness with constitutional symptoms associated with skin, glandular, respiratory, or gastrointestinal involvement. Tularemia usually can be treated effectively with streptomycin. Relapse most often occurs when patients are treated with bacteriostatic agents such as chloramphenicol or tetracycline. We present a case of ulceroglandular tularemia distinguished by its relapse after initial streptomycin/doxycycline therapy and subsequent slow response to additional streptomycin.

Orf: A Case Report, Brent W. Mohr, David Katz

Henry Ford Hospital Medical Journal

Ecthyma contagiosum (orf), a viral disease endemic to sheep and goats, can be zoonotically transmitted to humans through contact with infected animals. While the disease is common among sheep and goat herders worldwide, it comes to medical attention infrequently since it is generally a mild self-limited illness. We recently treated a healthy 36-year-old female who presented with bullous lesions on her fingers and a diffuse macular rash which had developed after antibiotic therapy for her bullae. She had been caring for her neighbor's goats which had recently been ill with "sore mouth." We discuss this case of orf and review …

Early Detection Of Hereditary Medullary Thyroid Cancer With Polymorphic Dna Probes, Steven A. Narod, Hagay Sobol, Isabelle Schuffenecker, R. Alan B. Ezekowitz, Gilbert M. Lenoir

Henry Ford Hospital Medical Journal

We have performed linkage analysis on 32 families with hereditary medullary thyroid cancer (MTC) wilh seven polymorphic DNA probes situated near the centromere of chromosome 10. Nineteen of these families were affected with multiple endocrine neoplasia type 2A (MEN 2A), and the remainder had MTC without pheochromocytoma. There were no instances of recombination between the MEN 2 A susceptibility gene and the IRBP.H4 marker. Two other probes, TBM.34 and MCK2, are also tightly linked(θ = 0.00 and θ = 0.02. respectively). Because TBM.34 and IRBP.H4/MCK2 are situated on opposite sides of the MEN 2A gene, screening with flanking DNA markers …

Genetic Mechanisms Of Neoplasia In Men 2, Charles E. Jackson, Robert A. Norum

Henry Ford Hospital Medical Journal

Several possible mechanisms for the initiation and progression of tumors in multiple endocrine neoplasia type 2 (MEN 2) merit consideration. Localization of MEN2A to the pericentromeric area of chromosome 10 indicates the site of the initial mutagenic event but does not explain the tissue specificity observed. The consistency of tissue involvement within families, despite the variability between families, suggests that the tumors result from separate but contiguous tissue-specific genes arranged in a particular linear order. Linkage studies in MEN 2A and 2B families are compatible with this contiguous gene theory. Data suggest that Knudson's two-mutational-event theory is applicable in MEN2, …

In Vitro Evaluation Of Cancer Patient Immune Responses Following Infusion Of Radiolabeled Murine Monoclonal Antibody, Kelly A. King

Loma Linda University Electronic Theses, Dissertations & Projects

Several monoclonal antibody (MAb) types derived from mice have been developed specifically for the use of targeting human tumors. The specificity of these murine MAb’s for their respective tumor antigens is very high making them potentially good immunotherapeutic agents for cancer treatment and diagnosis. A limiting factor in using these murine MAb’s in patients is the development of human anti-mouse antibody (HAMA). The presence of HAMA may reduce the effectiveness of MAb for tumor targeting. If patient sensitization could be quickly detected following the initial infusion of MAb, then future infusion could be altered or terminated to prevent further sensitization. …

Autonomic Nervous System And Cholesterol Transport, Yuan-Line Hung

Loma Linda University Electronic Theses, Dissertations & Projects

The effect of autonomic nervous system on cholesterol transport is of clinical interest because the relationship between lipoprotein metabolism and atherogenesis is expressed in coronary artery disease.

Generally speaking, beta blockers increase serum VLDL level but decrease both HDL and HDL₂ cholesterol levels without affecting the LDL in humans. We are unaware of reports concerning the effects of metoprolol on lipoprotein metabolism nor comparison of the effects of metoprolol and propranolol in the unhandled rat model. In part I we employed the unhandled rats to compared the effects of these 2 drugs administered with the drinking water in order …

Regeneration Of Meissner Corpuscles In Diabetic Mice Following Median Nerve Crush, Bonnie J. Forrester

Loma Linda University Electronic Theses, Dissertations & Projects

The hyperglycemic metabolic condition found in diabetes mellitus leads to the development of sensory neuropathies and causes delayed and incomplete neuronal regeneration. Diabetic sensory neuropathy has been shown to contribute to the impairment of light touch and low frequency vibratory perception. It is proposed that the regeneration of receptors that detect these sensations will be delayed and incomplete in diabetic animals with uncontrolled hyperglycemia. This study addresses the effects of diabetes on the regeneration of Meissner corpuscles, distal sensory endings that perceive light touch and low frequency vibration.

Corpuscles from 60 diabetic mice, and their age-matched, non-diabetic littermates, were examined …