Medicine and Health Sciences Commons^™

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

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.

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 …

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.

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 …

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.

Micronuclear Genome Organization In Euplotes Crassus: A Transposonlike Element Is Removed During Macronuclear Development, Scott Everet Baird, Gina M. Fino, Susan L. Tausta, Lawrence A. Klobutcher

Biological Sciences Faculty Publications

After mating, hypotrichous ciliated protozoa transform a set of their micronuclear chromosomes into thousands of short, linear DNA molecules that form the macronuclear genome. To examine micronuclear genome organization in the hypotrich Euplotes crassus, we have analyzed two cloned segments of micronuclear DNA as well as the macronuclear DNA molecules that are derived from them. E. crassus was found to display a number of features characteristic of other hypotrich genomes, including (i) clustering and close spacing of the precursors of macronuclear DNA molecules, (ii) the frequent occurrence of internal eliminated sequences within macronuclear precursors, (iii) overlapping macronuclear precursors, (iv) lack …

Bulletin 4133 - Fungal Diseases Of Pasture Legumes In Western Australia, M. J. Barbetti

Bulletins 4000 -

Subterranean clover (Trifolium subterraneum L.) is the most important annual pasture legume in Western Australia, forming the basis of some 6.5 million ha of annual pastures. The success of subterranean clover may be attributed to its ability to increase soil fertility as a consequence of nitrogen fixed, to withstand heavy grazing, to control soil erosion and, with some cultivars, to tolerate waterlogging. Fungal diseases have been a major limitation to the productivity of subterranean clover, affecting both the amount of plant matter and the quantity of seed produced.

Annual medic (Medicago spp., in particular M. polymorpha L.) is also an …

Chromosomal Localization Of Nucleic Acid-Binding Proteins By Affinity Mapping: Assignment Of The Ire-Binding Protein Gene To Human Chromosome 9, Matthias W. Hentze, Hector Seuanez, Stephen J. O'Brien, Joe B. Harford, Richard D. Klausner

Biology Faculty Articles

Three human mRNAs are regulated post-transcriptionally by iron via iron-responsive elements (IREs) contained in each mRNA. A cytoplasmic protein (IRE-BP) binds to these cis-acting elements and mediates the translational regulation of ferritin H- and L-chain mRNA and the iron-dependent stability of transferrin receptor (TfR) mRNA. We have taken advantage of the different mobilities of the human and rodent IRE/IRE-BP complexes on non-denaturing polyacrylamide gels to determine the chromosomal localization of the gene encoding the IRE-BP. Utilizing a panel of 34 different human/rodent hybrid cell lines we have assigned the IRE-BP gene to human chromosome 9. This new technique based on …

The Effect Of An Anti-Osteoclastic Agent And A Collagen Sponge On Root Resorption Following Regenerative Periodontal Surgery In The Dog, Rudolf Hahn

Loma Linda University Electronic Theses, Dissertations & Projects

Previous studies indicate that root resorption is a frequent sequela to significant amounts of periodontal repair following experimental regenerative periodontal surgical procedures in animal models. To study if root resorption can be reduced or prevented, periodontal repair was evaluated in large horizontal circumferential defects in the dog in the absence of influence from the oral environment. Diphosphonate (Cl₂ MDP) applied topically to the root surfaces prior to wound closure as well as the use of a collagen sponge membrane (Helistat®) draped over the root surfaces and a control with no additional treatment after defect creation were the modalities employed. …

Effect Of Photoperiod On Developmental Morphology And Enolase Isoenzyme Immunohistochemistry In Rat And Djungarian Hamster Superficial Pineal Glands, Chalmer D. Mcclure

Loma Linda University Electronic Theses, Dissertations & Projects

The best understood functional activity of the pineal gland is its diurnal production of melatonin in response to environmental lighting cues. Several enzymes of the melatonin pathway respond to daily photoperiod changes, for example hydroxyindole-O-methyltransferase (HIOMT) and serotonin N-acetyltransferase (SNAT). Increased levels of the glycolytic enzyme neuron-specific enolase (NSE) are thought to reflect increased physiological demands placed on neurons and neuroendocrine tissues. Homodimer non-neuronal enolase isoenzyme (NNE) is immunolocalized to cells, and the hybrid enolase (consisting of subunits from NSE and NNE) has been seen in cerebellar stellate and basket cells. Although not rate limiting, concentrations of both NSE and …

Santa Clara Magazine, Volume 31 Number 4, Summer 1989, Santa Clara University

Santa Clara Magazine

10 - COMPETITION AND CHANGE The way we organize human affairs is undergoing its most profound transformation in 2,000 years. By Thomas J. Peters

15 - WHAT'S A CHRISTIAN FAMILY TO DO? Some practical suggestions on family life in today's too busy world. By Mitch Finley '73

18 - REFLECTIONS OF A JESUIT'S EARLY YEARS Santa Clara's former president writes movingly of his experiences as a Jesuit novice. By William J. Rewak, SJ.

24 - A TOUCH OF WRY B.T. Collins '70 is an outrageously funny man who just can't seem to leave politics alone. By Barbara Wyman

28 - …

Mounting Sex Pheromone: A Novel Pheromone Responsible For Mate Recognition In The Ixodidae, Jgc Hamilton

Theses and Dissertations in Biomedical Sciences

Cholesterol oleate was demonstrated to be the cuticular contact sex pheromone of Dermacentor variabilis. This pheromone which has been termed the Mounting Sex Pheromone (MSP) was also demonstrated to be present on the surface of D. andersoni, Amblyomma maculatum and A. americanum. This contact sex pheromone enables males excited and attracted by 2,6-dichlorophenol to identify the female as a potential mating partner. The MSP is the second in the series of three sex pheromones guiding the hierarchy of behavioral responses which constitute tick courtship behavior. Tests with D. variabilis and D. andersoni showed that this pheromone …

The Composition And Concentrations Of Cyanobacteria In The North Landing River, Virginia And Upper Currituck Sound, North Carolina, Sonja E. Midgette

Biological Sciences Theses & Dissertations

A 12 month study of cyanobacteria composition and concentrations in the North Landing River and upper Currituck Sound was conducted from March 1986 to February 1987. Epifluorescence microscopy and the fluorochrome proflavin were used to determine cell concentrations and vertical, station to station and seasonal distribution of these cells. Cyanobacterial assemblage patterns were coupled with pH, temperature, salinity and water transparency readings.

Cyanobacteria were found to be a persistent and prolific phytoplankton component of both the river and upper sound. For a comparison of vertical distribution for each station, an analysis of variance for. two groups of equal size was …