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1992

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Full-Text Articles in Life Sciences

Purification And Ligand Binding Of A Soluble Class I Mhc Molecule Consisting Of The First Three Domains Of H-2kd Fused To B2-Microglobulin Expressed In The Baculovirus/Insect Cell System, Francois Godeau, Immanuel F. Luescher, David M. Ojcius, Cecile Saucier, Estelle Mottez, Lucien Cabanie, Philippe Kourilsky Dec 1992

Purification And Ligand Binding Of A Soluble Class I Mhc Molecule Consisting Of The First Three Domains Of H-2kd Fused To B2-Microglobulin Expressed In The Baculovirus/Insect Cell System, Francois Godeau, Immanuel F. Luescher, David M. Ojcius, Cecile Saucier, Estelle Mottez, Lucien Cabanie, Philippe Kourilsky

All Dugoni School of Dentistry Faculty Articles

A recombinant baculovirus encoding a single-chain murine major histocompatibility complex class I molecule in which the first three domains of H-2Kd are fused to beta 2-microglobulin (beta 2-m) via a 15-amino acid linker has been isolated and used to infect lepidopteran cells. A soluble, 391-amino acid single-chain H-2Kd (SC-Kd) molecule of 48 kDa was synthesized and glycosylated in insect cells and could be purified in the absence of detergents by affinity chromatography using the anti-H-2Kd monoclonal antibody SF1.1.1.1. We tested the ability of SC-Kd to bind antigenic peptides using a direct binding assay based on photoaffinity labeling. The photoreactive derivative …


Effects Of Dietary Calcium And Fasting On Osteoclast Recession And Recruitment In Calcium Deficient Rats, Kenneth R. Wright Dec 1992

Effects Of Dietary Calcium And Fasting On Osteoclast Recession And Recruitment In Calcium Deficient Rats, Kenneth R. Wright

Loma Linda University Electronic Theses, Dissertations & Projects

The osteoclast, a multinucleated giant cell, has as its role the resorption of bone matrix with consequent release of calcium. The osteoclast is thought to derive from a pluripotent stem cell which also gives rise to monocytes and macrophages. The development, activation, and inhibition of osteoclasts are influenced by a number of systemic and local factors. The release of these factors is influenced by changes in the dietary status of the animal. Calcium deficiency is known to stimulate the recruitment and activity of large numbers of osteoclasts. These osteoclasts can subsequently be inhibited by increasing dietary calcium intake. Disappearance of …


The Effect Of Trimethyltin On The Cholinergic System Of The Rat Hippocampus, Richard L. Cannon Dec 1992

The Effect Of Trimethyltin On The Cholinergic System Of The Rat Hippocampus, Richard L. Cannon

Electronic Theses and Dissertations

Trimethyltin (TMT) is a neurotoxin occurring in the environment. Exposure to (TMT) is known to destroy specific neuronal components of the hippocampus in the rat and to cause clinical symptoms in exposed humans, including mnemonic deficits, that indicate hippocampal involvement. In addition to hippocampal cell loss TMT causes significant increases in cholinergic markers such as acetylcholinesterase (AChE) stain density and choline acetyltransferase (ChAT) activity in the hippocampus of rats. However, despite these observations the effect of TMT on hippocampal cholinergic system has not been investigated in detail. The purpose of the present study was to elucidate more fully the consequences …


Neurophysiologic Bases For Abnormal Muscle Coactivation Patterns In Hemiparetic Stroke, Julius P.A. Dewald Dec 1992

Neurophysiologic Bases For Abnormal Muscle Coactivation Patterns In Hemiparetic Stroke, Julius P.A. Dewald

Loma Linda University Electronic Theses, Dissertations & Projects

To study disturbed muscle activation patterns in hemiparetic stroke, the EMG activity in spastic-paretic and contralateral elbow muscles of 10 stroke subjects were compared during graded directionally varying voluntary isometric contractions. We observed significant shifts in the peak EMG angle and increases in the angular range of EMG activity in the impaired upper limb. Furthermore, we observed stereotypic coactivation of many muscles acting at the same joint or at different joints across all subjects.

The effect of elbow angle changes on muscle coactivation patterns was studied by examining isometric torque-angle relations under passive and active conditions in 8 stroke subjects. …


Identification And Characterization Of Possible Multiple Binding Sites For ((3)H)8-Oh-Dpat In The Hippocampus, Dawna Lea Evans Dec 1992

Identification And Characterization Of Possible Multiple Binding Sites For ((3)H)8-Oh-Dpat In The Hippocampus, Dawna Lea Evans

Masters Theses

No abstract provided.


Evidence For The Alternative Pathway Of Complement Activation In The Nurse Shark, Lieneke Cecile Culbreath Nov 1992

Evidence For The Alternative Pathway Of Complement Activation In The Nurse Shark, Lieneke Cecile Culbreath

FIU Electronic Theses and Dissertations

Complement is activated via two pathways: classical (CCP) and alternative (ACP). The CCP has been demonstrated in the nurse shark. The ACP has not been demonstrated in any cartilagenous fish. Nurse shark serum was evaluated for complement activity by its ability to lyse heterologous erythrocytes. As CCP activity requires calcium and magnesium, activity of shark serum chelated with EGTA (a selective calcium chelator) or EDTA (a chelator of calcium and magnesium) was assessed. Activity remained in serum chelated with EGTA but not EDTA. Furthermore, activity of chelated serum was enhanced by added magnesium. Activation of shark complement by activators of …


1992 Scholars And Artists Bibliography, Michael Schwartz Library, Cleveland State University, Friends Of The Michael Schwartz Library Oct 1992

1992 Scholars And Artists Bibliography, Michael Schwartz Library, Cleveland State University, Friends Of The Michael Schwartz Library

Scholars and Artists Bibliographies

This bibliography was created for the annual Friends of the Michael Schwartz Library Scholars and Artists Reception, recognizing scholarly and creative achievements of Cleveland State University faculty, staff and emeriti


Identification And Prevalence Of A Genetic Defect That Causes Leukocyte Adhesion Deficiency In Holstein Cattle, Dale E. Shuster, Marcus E. Kehrli Jr., Mark R. Ackermann, R. O. Gilbert Oct 1992

Identification And Prevalence Of A Genetic Defect That Causes Leukocyte Adhesion Deficiency In Holstein Cattle, Dale E. Shuster, Marcus E. Kehrli Jr., Mark R. Ackermann, R. O. Gilbert

Mark R. Ackermann

Two point mutations were identified within the gene encoding bovine CD18 in a Holstein calf afflicted with leukocyte adhesion deficiency (LAD). One mutation causes an aspartic acid to glycine substitution at amino acid 128 (D128G) in the highly conserved extracellular region of this adhesion glycoprotein, a region where several mutations have been found to cause human LAD. The other mutation is silent. Twenty calves with clinical symptoms of LAD were tested, and all were homozygous for the D128G allele. In addition, two calves homozygous for the D128G allele were identified during widespread DNA testing, and both were subsequently found to …


Santa Clara Magazine, Volume 35 Number 1, Fall 1992, Santa Clara University Oct 1992

Santa Clara Magazine, Volume 35 Number 1, Fall 1992, Santa Clara University

Santa Clara Magazine

11 - SENIOR SEMINAR Elderhostel brings a different kind of student to Santa Clara. By Christine Spielberger '69

14 - DAYS RICH WITH EMPTINESS A noted Catholic writer reflects on his annual retreat to a Trappist abbey, a respite from the daily stress of getting and spending. In a related article, he discusses "The Inner Experience," an unpublished book by Thomas Merton that examines the modern contemplative lifestyle. By Mitch Finley '73

22 - WHAT'S WRONG WITH THIS PICTURE? The image of an all -male Senate committee weighing the merits of Anita Hill 's charges against Clarence Thomas spurred a …


Ua12/12 Your Healthstyle, Wku Wellness Center Oct 1992

Ua12/12 Your Healthstyle, Wku Wellness Center

WKU Archives Records

Newsletter created by the Wellness Center regarding services, health and fitness.


Changes In Intracellular Chloride During Osmotic Stress And L-Alanine Uptake In Mouse Hepatocytes, Kening Wang Oct 1992

Changes In Intracellular Chloride During Osmotic Stress And L-Alanine Uptake In Mouse Hepatocytes, Kening Wang

Electronic Theses and Dissertations

A stable intracellular ionic environment is necessary for hepatocytes to function normally. Thus, during hypotonic shock or L-alanine uptake, hepatocytes swell and then exhibit a regulatory volume decrease (RVD), which comprises an increase in K$\sp+$ conductance (G$\sb{\rm K}$), an increased K$\sp+$ efflux, and a hyperpolarization of transmembrane potential (V$\sb{\rm m}$). Since hepatocyte intracellular Cl$\sp-$ has been demonstrated to distribute passively with V$\sb{\rm m}$, this study is designed to test the hypothesis that the hypotonic shock- or L-alanine uptake-induced hyperpolarization of V$\sb{\rm m}$ might provide an electromotive force for the efflux of hepatocyte intracellular Cl$\sp-$, which in turn would contribute osmotically …


The Effects Of Post-Event Massage Therapy On Multistage Ultraendurance Cycling, Tracey Hodges Drews Oct 1992

The Effects Of Post-Event Massage Therapy On Multistage Ultraendurance Cycling, Tracey Hodges Drews

Human Movement Studies & Special Education Theses & Dissertations

Post-event massage therapy has been proposed to hasten muscle recovery and improve ensuing performance in multistage ultraendurance cycling events. Six elite male cyclists competed in two 4·d stage races of 161 kmd·1 on a computerized race simulator over challenging terrain in a controlled laboratory environment with an average completion time of 275 ± 28 min. In a crossover experimental design, subjects received either 30 min post-event massage therapy or a 30 min inactivated diathermy treatment serving as a blind placebo after each 161 km race. The subjects repeated the 4·d, 161 km·d·1 event 18d later receiving alternate post-event …


Red Butte Canyon Research Natural Area: History, Flora, Geology, Climate, And Ecology, James R. Ehleringer, Lois A. Arnow, Ted Arnow, Irving B. Mcnulty, Norman C. Negus Sep 1992

Red Butte Canyon Research Natural Area: History, Flora, Geology, Climate, And Ecology, James R. Ehleringer, Lois A. Arnow, Ted Arnow, Irving B. Mcnulty, Norman C. Negus

Great Basin Naturalist

Red Butte Canyon is a protected, near pristine canyon entering Salt Lake Valley, Utah. It contains a well-developed riparian zone and a perennial stream; hillside vegetation ranges from grasslands on the lower limits to Douglas-fir and aspen stands at the upper elevations. In this paper we describe the history of human impact, natural history aspects of climate, geology, and ecology, and faunal and floral information for key species in the canyon. The role and importance of Research Natural Areas is discussed, particularly with respect to the need to protect Red Butte Canyon—one of the few remaining undisturbed riparian ecosystems in …


Sublocalization Of The Multiple Endocrine Neoplasia Type 1 Gene, Catharina Larsson, Gunther Weber, Marie Janson Sep 1992

Sublocalization Of The Multiple Endocrine Neoplasia Type 1 Gene, Catharina Larsson, Gunther Weber, Marie Janson

Henry Ford Hospital Medical Journal

Tumorigenesis in multiple endocrine neoplasia type 1 (MEN 1) involves the unmasking of a recessive mutation at the MEN 1 locus which has been mapped to chromosomal region 11q11-13. By analyzing 58 DNA markers on a panel of radiation-reduced somatic cell hybrids, the region encompassing the MEN 1 gene was divided into nine subregions. Pulsed field gel electrophoresis analysis of markers within subgroups showed that the recombination rate around the MEN 1 locus is high. Combined linkage analysis in MEN 1 families and deletion mapping in MEN 1-related tumors suggest the MEN 1 gene is located centromeric to D11S807 and …


Proceedings Of The Fourth International Workshop On Multiple Endocrine Neoplasia: Introduction, Robert F. Gagel, Charles E. Jackson Sep 1992

Proceedings Of The Fourth International Workshop On Multiple Endocrine Neoplasia: Introduction, Robert F. Gagel, Charles E. Jackson

Henry Ford Hospital Medical Journal

No abstract provided.


The Importance Of Screening For The Men 1 Syndrome: Diagnostic Results And Clinical Management, C. J. M. Lips, H. P. F. Koppeschaar, M. J. H. Berends, J. M. Jansen-Schillhorn Van Veen, A. Struyvenberg, Th. J. M. V. Van Vroonhoven Sep 1992

The Importance Of Screening For The Men 1 Syndrome: Diagnostic Results And Clinical Management, C. J. M. Lips, H. P. F. Koppeschaar, M. J. H. Berends, J. M. Jansen-Schillhorn Van Veen, A. Struyvenberg, Th. J. M. V. Van Vroonhoven

Henry Ford Hospital Medical Journal

No abstract provided.


Prospective Screening In Multiple Endocrine Neoplasia Type 1, Britt Skogseid, Kjell Oberg Sep 1992

Prospective Screening In Multiple Endocrine Neoplasia Type 1, Britt Skogseid, Kjell Oberg

Henry Ford Hospital Medical Journal

To assess the age of clinically detectable onset of multiple endocrine neoplasia type 1 (MEN 1), 88 members of four families were invited to participate in a ten-year biochemical screening program. Evidence for clinically detectable MEN 1 was found in adolescence. Pancreatic endocrine dysfunction constituted the presenting lesion in a majority of these individuals. The age at diagnosis of pancreatic endocrine tumors averaged 25 years and was lowered by almost two decades by prospective investigation. Furthermore, the penetrance of the pancreatic endocrine and parathyroid lesions equaled the penetrance found in autopsy studies. The use of a standardized meal stimulation test …


Practical Guidelines For Dna-Based Testing In Multiple Endocrine Neoplasia Type 1, Catharina Larsson, Magnus Nordenskjold, Britt Skogseid, Kjell Oberg Sep 1992

Practical Guidelines For Dna-Based Testing In Multiple Endocrine Neoplasia Type 1, Catharina Larsson, Magnus Nordenskjold, Britt Skogseid, Kjell Oberg

Henry Ford Hospital Medical Journal

Multiple endocrine neoplasia type 1 (MEN 1) is an autosomal dominant predisposition to neoplastic lesions of the parathyroid glands, the neuroendocrine pancreas, and the anterior pituitary gland. The predisposing genetic defect was localized to the long arm of chromosome 11 by genetic linkage analysis in three affected families. By analyzing six MEN 1 families with 14 DNA marker systems located close to the MEN 1 gene, we have developed a method to identify carriers of the MEN 1 predisposition. We describe practical aspects of such DNA-based diagnostic procedures.


Hyperparathyroidism With Normal Albumin-Corrected Total Calcium In Patients With Multiple Endocrine Neoplasia Type 1, J. J. Shepherd, Bin Tean Teh, V. Parameswaran, R. David Sep 1992

Hyperparathyroidism With Normal Albumin-Corrected Total Calcium In Patients With Multiple Endocrine Neoplasia Type 1, J. J. Shepherd, Bin Tean Teh, V. Parameswaran, R. David

Henry Ford Hospital Medical Journal

In the largest reported family of patients with multiple endocrine neoplasia type 1 (MEN 1), hyperparathyroidism was expressed at first screening in 33 patients by elevation of ionized calcium (IC) (30 cases) or parathyroid hormone (three cases) without elevation of albumin-corrected total calcium (ACTC). Three of these 33 patients have shown a progressive rise in IC and later an elevation of ACTC. However, the age distribution suggests that in others the level of IC may remain stable at a minimally elevated level throughout life with ACTC remaining normal except for transient rises at the times of intercurrent illness or surgical …


Parathyroid Tumor Biology In Familial Multiple Endocrine Neoplasia Type 1: A Model For Cancer Development, Maria Luisa Brandi Sep 1992

Parathyroid Tumor Biology In Familial Multiple Endocrine Neoplasia Type 1: A Model For Cancer Development, Maria Luisa Brandi

Henry Ford Hospital Medical Journal

Familial multiple endocrine neoplasia type 1 (FMEN 1) is an autosomal dominant disorder characterized by tumors of the parathyroid glands, pancreatic islets, and anterior pituitary. Hyperplasia appears to be the typical histopathological lesion in FMEN 1 endocrine tumors. A circulating mitogen related to basic fibroblast growth factor was active on proliferation of clonal bovine and human parathyroid endothelial cells. Moreover, the FMEN 1 mitogen modulated differentiation of human parathyroid endothelial cell in vitro. All these facts suggested that an extrinsic factor was active on parathyroid endothelial cell growth and differentiation. The FMEN 1 gene maps to chromosome 11q13, and allelic …


Surgical Treatment Of The Endocrine Pancreas And Zollinger-Ellison Syndrome In The Men 1 Syndrome, Norman W. Thompson Sep 1992

Surgical Treatment Of The Endocrine Pancreas And Zollinger-Ellison Syndrome In The Men 1 Syndrome, Norman W. Thompson

Henry Ford Hospital Medical Journal

Islet cell neoplasia is a frequent occurrence in multiple endocrine neoplasia type 1 (MEN 1). Sixteen of 27 patients with MEN 1 developed functioning endocrine pancreatic tumor syndromes. Eleven of the 16 developed Zollinger-Ellison syndrome and each was evaluated by a combination of computed tomography and hepatic angiography to exclude hepatic metastasis and percutaneous transhepatic catheterization to localize the tumor. Seven of the 11 patients were found to have duodenal gastrinomas with multiple duodenal tumors in three patients. Four of the 11 patients had only pancreatic gastrinomas. In addition to the gastrinomas, other types of islet tumors in the pancreatic …


Isolation Of Yac Clones From The Pericentromeric Region Of Chromosome 10 And Development Of New Genetic Markers Linked To The Multiple Endocrine Neoplasia Type 2a Gene, Terry C. Lairmore, James R. Howe, Shenshen Dou, Rosalie Veile, Jennifer A. Korte-Sarfaty, Samuel A. Wells Jr., Helen Donis-Keller Sep 1992

Isolation Of Yac Clones From The Pericentromeric Region Of Chromosome 10 And Development Of New Genetic Markers Linked To The Multiple Endocrine Neoplasia Type 2a Gene, Terry C. Lairmore, James R. Howe, Shenshen Dou, Rosalie Veile, Jennifer A. Korte-Sarfaty, Samuel A. Wells Jr., Helen Donis-Keller

Henry Ford Hospital Medical Journal

Genetic linkage mapping and contig assembly using yeast artificial chromosome (YAC) technology form the basis of our strategy to clone and define the genomic structure of the pericentromeric region of chromosome 10 containing the multiple endocrine neoplasia type 2A gene. Thus far YAC walks have been initiated from five chromosome 10 pericentromeric loci including RBP3, D10S94, RET, D10Z1, and FNRB. Long range pulsed-field gel electrophoresis maps are constructed from the YACs isolated to define clone overlaps and to identify putative CpG islands. Bidirectional YAC walks are continued by rescreening the YAC library with sequence-tagged site assays developed from endclones. Several …


A Preliminary Analysis Of Consortium Data For Markers Tightly Linked To Multiple Endocrine Neoplasia Type 2a, J. B. Lichter, S. M. Hackleman, B. A. J. Ponder, D. Easton, S. A. Narod, G. M. Lenoir, R. F. Gagel, N. E. Simpson, E. Gardner, P. J. Goodfellow, S. Takai, A. J. Pakstis, K. K. Kidd Sep 1992

A Preliminary Analysis Of Consortium Data For Markers Tightly Linked To Multiple Endocrine Neoplasia Type 2a, J. B. Lichter, S. M. Hackleman, B. A. J. Ponder, D. Easton, S. A. Narod, G. M. Lenoir, R. F. Gagel, N. E. Simpson, E. Gardner, P. J. Goodfellow, S. Takai, A. J. Pakstis, K. K. Kidd

Henry Ford Hospital Medical Journal

We have analyzed DNA marker typing data contributed by six independent groups to estimate the pairwise genetic distances between these markers and the locus for multiple endocrine neoplasia type 2A (MEN 2A). We used LIPED to calculate these distances for female, male, and sex-average linkage maps and to determine the corresponding LOD scores. The preliminary analyses of this large data set (89 MEN 2A families and five non-MEN 2A references families, with 1,934 total individuals) are reported here. These refined estimates of the genetic map in this region will aid in the assignment of presymptomatic diagnoses. This study clearly points …


Localization Of The Gene For Men 2a, Jay B. Lichter, Michael Difilippantonio, Jingshi Wu, Diane Miller, David C. Ward, Paul J. Goodfellow, Kenneth K. Kidd Sep 1992

Localization Of The Gene For Men 2a, Jay B. Lichter, Michael Difilippantonio, Jingshi Wu, Diane Miller, David C. Ward, Paul J. Goodfellow, Kenneth K. Kidd

Henry Ford Hospital Medical Journal

The search for the gene that causes the multiple endocrine neoplasia type 2A (MEN 2A) syndrome is entering a new phase. Genetic linkage studies have localized the gene to the pericentromeric region of chromosome 10. The statistical portion of mapping the gene for MEN 2A is nearly complete and now classical molecular biological/gene mapping techniques will be employed. We have used fluorescence in situ hybridization to estimate the size of the MEN2A region to be about 2 to 5 mb, using some liberal assumptions; at worst the region should contain no more than about 10 mb of non-alphoid DNA. Our …


Screening For Multiple Endocrine Neoplasia Type 2a With Dna-Polymorphism Analysis, Estelle M.-F. Lamothe, Steven A. Narod, Shari Miller, Paul J. Goodfellow, David E. C. Cole, Dawna Gilchrist, Zdenka Pausova, David Goltzman, Geoffrey N. Hendy Sep 1992

Screening For Multiple Endocrine Neoplasia Type 2a With Dna-Polymorphism Analysis, Estelle M.-F. Lamothe, Steven A. Narod, Shari Miller, Paul J. Goodfellow, David E. C. Cole, Dawna Gilchrist, Zdenka Pausova, David Goltzman, Geoffrey N. Hendy

Henry Ford Hospital Medical Journal

Nine chromosome 10 DNA markers (FNRB, D10S34, D10Z1, MEN203, D10S94, RBP3, D10S15, MBP [48.11], D10S22) were typed in two large Canadian pedigrees with multiple endocrine neoplasia type 2A (MEN 2A). These markers and the gene for MEN 2A (MEN2A) are believed to be in one linkage group spanning approximately 15 cM (male). MEN203 and D10S94 were informative and tightly linked to MEN2A with no recombinants observed in 26 meiotic events. D10S15 (MCK2), widely used in DNA genotyping predictions, demonstrated two recombinants in these two families. The use of multiple flanking markers increases both the likelihood of informativeness and the accuracy …


High-Sensitivity Serum Calcitonin Assays Applied To Screening For Thyroid C-Cell Disease In Multiple Endocrine Neoplasia Type 2a, Michael M. Kaplan, Glenn M. Stall, Timothy Cummings, Andrew Macaulay, Philippe Motté, Hubert J. Wolfe, Seymour Reichlin, Arman J. Tashjian Jr. Sep 1992

High-Sensitivity Serum Calcitonin Assays Applied To Screening For Thyroid C-Cell Disease In Multiple Endocrine Neoplasia Type 2a, Michael M. Kaplan, Glenn M. Stall, Timothy Cummings, Andrew Macaulay, Philippe Motté, Hubert J. Wolfe, Seymour Reichlin, Arman J. Tashjian Jr.

Henry Ford Hospital Medical Journal

Two serum calcitonin assays with sensitivities ≤ 10 pg/mL were compared to our standard radioimmunoassay (sensitivity 100 pg/mL) in multiple endocrine neoplasia type 2A (MEN 2A) screening. Values from the Nichols displacement radioimmunoassay averaged 38% higher than values from the CIS immunoradiometric assay; values from both were highly correlated, r = 0.845. In three individuals, both of the newer assays revealed abnormalities in pentagastrin tests three to four years before abnormalities were detected by the standard assay. Pentagastrin tests after total thyroidectomy were assayed by the newer methods in patients with medullary thyroid carcinoma (MTC) diagnosed at initial testing (group …


Genetics Of The Multiple Endocrine Neoplasia Type 2b Syndrome, Charles E. Jackson, Robert A. Norum Sep 1992

Genetics Of The Multiple Endocrine Neoplasia Type 2b Syndrome, Charles E. Jackson, Robert A. Norum

Henry Ford Hospital Medical Journal

Multiple endocrine neoplasia type 2B (MEN 2B) is similar to MEN 2A in that both autosomal dominant syndromes include medullary thyroid cancers and pheochromocytomas. It is distinct in that MEN 2B patients have much earlier age of onset with more aggressive tumors and mucosa/neuromas of the lips and tongue. The neuromas allow ascertainment generally before age 5. Studies of two and three generations of 14 MEN 2B families disclosed close linkage of the MEN 2B gene to DNA markers to which MEN2A had been linked. Multipoint analysis utilizing additional results in three generations of a 15th family have disclosed a …


Multiple Endocrine Neoplasia Type 2b: Eighteen-Year Follow-Up Of A Four-Generation Family, Glen W. Sizemore, J. Aiden Carney, Hossein Gharib, Charles C. Capen Sep 1992

Multiple Endocrine Neoplasia Type 2b: Eighteen-Year Follow-Up Of A Four-Generation Family, Glen W. Sizemore, J. Aiden Carney, Hossein Gharib, Charles C. Capen

Henry Ford Hospital Medical Journal

Seven members with multiple endocrine neoplasia type 2B from a 15-member family have been followed for 18 years. All affected had the neuroma phenotype in a distribution compatible with autosomal dominant inheritance. The phenotype features have allowed 100% initial and continuing prediction of affected versus nonaffected status in as early as 1.5 years. Among the affected: immunoreactive plasma calcitonin (iCT) concentration was high in 100%; thyroid palpation was false-negative in 71%; and thyroid scintiscan was false-negative in 83%. All had total thyroidectomy, plus lymphadenectomy in three, for bilateral medullary thyroid carcinoma (MTC) or C-cell hyperplasia (in the youngest). None has …


Long-Term Follow-Up In Four Large Men 2 Families In The Netherlands, C. J. M. Lips, M. J. H. Berends, J. Brouwers-Smalbraak, R. A. Geerdink, W. H. L. Hackeng, J. M. Jansen-Schillhorn Van Veen, A. Struyvenberg, Th. J. M. V. Van Vroonhoven Sep 1992

Long-Term Follow-Up In Four Large Men 2 Families In The Netherlands, C. J. M. Lips, M. J. H. Berends, J. Brouwers-Smalbraak, R. A. Geerdink, W. H. L. Hackeng, J. M. Jansen-Schillhorn Van Veen, A. Struyvenberg, Th. J. M. V. Van Vroonhoven

Henry Ford Hospital Medical Journal

Results of follow-up studies in four large multiple endocrine neoplasia type 2A families (total of 95 patients affected) have shown a positive effect on the course of the disease since early screening and intervention were initiated in 1974.


Characterization Of The Clinical Features Of Five Families With Hereditary Primary Cutaneous Lichen Amyloidosis And Multiple Endocrine Neoplasia Type 2, Marion F. Robinson, Eric J. Furst, Vincenzo Nunziata, Maria Luisa Brandi, Jorge P. Ferrer, Maria J. G. Martins Bugalho, Guiseppe Di Giovanni, Richard J. H. Smith, Donald T. Donovan, Bobby R. Alford, James F. Hejtmancik, Vittorio Colantuoni, Loredana Quadro, Edward Limbert, Irene Halperin, Enric Vilardell, Robert F. Gagel Sep 1992

Characterization Of The Clinical Features Of Five Families With Hereditary Primary Cutaneous Lichen Amyloidosis And Multiple Endocrine Neoplasia Type 2, Marion F. Robinson, Eric J. Furst, Vincenzo Nunziata, Maria Luisa Brandi, Jorge P. Ferrer, Maria J. G. Martins Bugalho, Guiseppe Di Giovanni, Richard J. H. Smith, Donald T. Donovan, Bobby R. Alford, James F. Hejtmancik, Vittorio Colantuoni, Loredana Quadro, Edward Limbert, Irene Halperin, Enric Vilardell, Robert F. Gagel

Henry Ford Hospital Medical Journal

The hereditary conditions of primary cutaneous lichen amyloidosis and multiple endocrine neoplasia type 2 (MEN 2) are rare clinical entities. The initial reports of two families in which the two conditions coincided have led to the identification of at least eight additional families with this clinical syndrome. In this report we describe the clinical features in five of these eight families. The salient feature in these five families is the presence of unilateral (46%) or bilateral (64%) pruritic and lichenoid skin lesions located over the upper portion of the back. Family members describe these skin lesions as intermittently intensely pruritic …