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Wermer syndrom

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https://www.readbyqxmd.com/read/27734708/-multiple-endocrine-neoplasia-i-wermers-syndrome-forms-of-clinical-manifestation-5-case-studies
#1
Karolína Drbalová, Kateřina Herdová, Petr Krejčí, Monika Nývltová, Svatopluk Solař, Lenka Vedralová, Pavel Záruba, David Netuka, Petr Bavor
Multiple Endocrine Neoplasia (MEN) is a condition in which several endocrine organs of an individual are affected by adenoma, hyperplasia and less often carcinoma, either simultaneously or at different stages of life. Two existing syndromes, MEN1 and MEN2 (2A, 2B), in literature is also mentioned MEN4, are associated also with other non-endocrine disorders. MEN1 (Wermer syndrome) affects the pituitary, parathyroid, and pancreatic area. 95 % of patients show very early manifestation of hyperparathyroidism, often before 40 years of age...
2016: Vnitr̆ní Lékar̆ství
https://www.readbyqxmd.com/read/25312840/the-value-of-ct-angiography-in-patients-with-acute-severe-headache
#2
I M E Alons, I R van den Wijngaard, R J Verheul, G Lycklama à Nijeholt, M J H Wermer, A Algra, K Jellema
Patients with acute severe headache may have a secondary form of headache. Standard head computer tomography (CT) and cerebrospinal fluid (CSF) examination are often performed in the absence of neurological deficits to exclude subarachnoid hemorrhage (SAH). Increasingly, patients undergo subsequent CT angiography (CTA) to exclude cerebral venous thrombosis (CVT), dissection or reversible cerebral vasoconstriction syndrome (RCVS). It is unknown whether this additional imaging increases diagnostic yield. We aimed to evaluate the yield of CTA in patients with acute severe headache with normal neurological examination and no abnormalities at standard CT and CSF analysis...
March 2015: Acta Neurologica Scandinavica
https://www.readbyqxmd.com/read/23569534/multiple-endocrine-neoplasia-type-1-presenting-multiple-lipomas-and-hypoglycemia-onset
#3
Jianzhong Li, Lixian Zeng, Yidong Yang, Yashi Zhan, Jin Tao, Bin Wu
BACKGROUND: Multiple endocrine neoplasia type 1 (MEN1), also called Wermer syndrome, is an autosomal dominant disorder characterized by tumors of the parathyroid glands, the anterior pituitary, and the endocrine pancreas. CASE REPORT: Here, we report a case of MEN1. Our patient was a 44-year-old woman who manifested typical features of MEN1, including insulinoma, pituitary tumors, and parathyroidoma, and exhibited multiple lipomas and a gastrinoma with duodenal ulcers...
2012: American Journal of Case Reports
https://www.readbyqxmd.com/read/23435440/diagnosis-and-treatment-of-multiple-endocrine-neoplasia-type-1-men1
#4
REVIEW
S Gaztambide, F Vazquez, L Castaño
Multiple endocrine neoplasia type 1 (MEN1; formerly known as Wermer syndrome) is a rare disorder characterized by the combined occurrence of two or more tumors involving parathyroid, pancreatic islets and anterior pituitary glands; some other tumors have also been described. In most cases it is inherited in an autosomic dominant manner but it may occur sporadically. The MEN1 gene (MEN1) is located on chromosome 11q13, it is composed of ten exons that encode a 610 amino acid protein called menin. Menin, with no homology to any other known protein, interacts with several different proteins and plays an important role in regulation of cell growth, cell cycle, genome stability and synapse plasticity...
March 2013: Minerva Endocrinologica
https://www.readbyqxmd.com/read/22199068/neurocutaneous-spectrum-of-multiple-endocrine-neoplasia-1
#5
Shireen Furtado, Nandita Ghosal, Sunil V Furtado, Kanchan Gupta, Alangar S Hegde
Multiple endocrine neoplasia type I or Wermer syndrome is characterized by primary hyperparathyroidism, enteropancreatic endocrine tumor, and a pituitary pathology. A 35-year-old male presented with visual field defects, hyperprolactinemia, and hypogonadism. He also had multiple infraumbilical skin-colored nodules. A syndromal association of Wermer syndrome was derived using the dermal, pituitary, parathyroid, and gastrointestinal hormonal manifestations of the tumor. The radiological and histological findings of lesion which underwent biopsy are discussed...
January 2012: Indian Journal of Dermatology, Venereology and Leprology
https://www.readbyqxmd.com/read/19380653/image-of-the-month-wermer-syndrome-and-zollinger-ellison-syndrome
#6
Timothy L Frankel, Paul G Gauger
No abstract text is available yet for this article.
April 2009: Archives of Surgery
https://www.readbyqxmd.com/read/19012220/bilateral-trapped-lung-with-concomitant-wermer-s-syndrome-and-cutis-laxa
#7
D Kilic, A Findikcioglu, E Sahin, A Bilen, O Bakiner, A Hatipoglu
We report a rare occurrence of trapped lung associated with Wermer's syndrome and cutis laxa. A 25-year-old man with bilateral trapped lung presented with respiratory distress. Additional investigation revealed adenoma of the parathyroid and hypophysis, a neuroendocrine abdominal tumor, and cutis laxa. Successful treatment of the respiratory symptoms was achieved through a median sternotomy with bilateral decortication, and excision of the bilateral fibrous pleural bands and bullae on the right upper lobe. Octreotide therapy was initiated and a percutaneous ultrasound-guided alcohol injection to the parathyroid adenoma was done...
December 2008: Thoracic and Cardiovascular Surgeon
https://www.readbyqxmd.com/read/17921680/a-37-year-old-married-woman-with-3-month-history-of-galactorrhoea-and-irregular-menstrual-cycle-diagnosis-multiple-endocrine-neoplasia-type-1-men-1-also-known-as-wermer-s-syndrome
#8
Chandan Jyoti Das, Jyotindu Debnath, N G Javan, Manash Pratim Baruah
No abstract text is available yet for this article.
September 2007: Annals of Saudi Medicine
https://www.readbyqxmd.com/read/17379178/-a-multiple-endocrine-neoplasia-type-1-observatory-in-a-french-speaking-area-a-tool-from-the-endocrine-tumor-study-group-gte
#9
REVIEW
P Goudet, C Bonithon, A Costa, G Cadiot, E Baudin, A Murat, B Delemer, A Tabarin, P Lecomte, A Calender
Wermer's syndrome or Multiple Endocrine Neoplasia Type-1 (MEN1) is an autosomal dominant inherited disease, related to mutations in MEN1, an approximately 10-kb gene encoding menin, localized on chromosome 11q13. The Endocrine Tumor Group (GTE) has set up a MEN1 observatory of 1001 regularly followed MEN1 cases. This observatory aims at registering and evaluating MEN1 cases in a large cohort. Any new study on a particular unexplored aspect of the disease may be proposed by a physician to the GTE. This article describes the way to diagnose a new MEN1 case and to register it...
June 2007: Annales D'endocrinologie
https://www.readbyqxmd.com/read/17342698/-men-type-1-and-chronic-renal-failure-a-rarely-reported-association
#10
C Molino, F Fabbian, G Russo, S Cantelli, A Bortot, A Galdi, L Catizone
BACKGROUND: Multiple endocrine neoplasia type 1 (MEN 1), or Wermer's syndrome, is a rare autosomal dominant genetic syndrome characterized by tumors or hyperplasia involving the pituitary, parathyroid, and pancreatic islet cells. Association between MEN 1 and nephrocalcinosis is well known, though data published in medical literature regarding Wermer's syndrome and chronic renal failure relation are still rare. CASE: A 70-year-old Caucasian female patient had a history of primitive hyperparathyroidism, prolactinoma, glucagonoma, adrenal adenoma and pulmonary neuroendocrine neoplasia...
January 2007: Giornale Italiano di Nefrologia: Organo Ufficiale Della Società Italiana di Nefrologia
https://www.readbyqxmd.com/read/16939409/vitamin-d-mediated-hypercalcemia-in-slack-skin-disease-evidence-for-involvement-of-extrarenal-25-hydroxyvitamin-d-1alpha-hydroxylase
#11
COMPARATIVE STUDY
Helen Karakelides, Jordan L Geller, Arnold L Schroeter, Hong Chen, Philip S Behn, John S Adams, Martin Hewison, Robert A Wermers
UNLABELLED: A case of granulomatous slack skin disease is presented in which we studied the possible involvement of extrarenal 1,25(OH)2D in the pathogenesis of the patient's hypercalcemia. Immunolocalization of 1alpha-OH in skin showed simultaneous dysregulation in epithelial and granulomatous cells. INTRODUCTION: Granuloma-forming diseases such as sarcoidosis are associated with extrarenal synthesis of active 1,25-dihydroxyvitamin D [1,25(OH)2D]. Here we describe a case of granulomatous slack skin disease in which we have studied the possible involvement of extrarenal synthesis of 1,25(OH)2D in the pathogenesis of the patient's hypercalcemia...
September 2006: Journal of Bone and Mineral Research: the Official Journal of the American Society for Bone and Mineral Research
https://www.readbyqxmd.com/read/16821356/-multiple-endocrine-neoplasia-type-1-a-possible-cause-of-severe-and-recurrent-urolithiasis
#12
Lamine Niang, Mohamed Amine Lackmichi, Sophie Perie, Frédéric Thibault, Bernard Gattegno, Philippe Thibault, Olivier Traxer
Multiple endocrine neoplasia (MEN) is defined by the presence of at least two functionally unrelated endocrine gland tumours in the same subject. There are three types of MEN. MEN type 1 (or Wermer syndrome) is an autosomal dominant cancer syndrome. In the light of a case report, the authors recall the characteristics of MEN type 1 responsible for severe and recurrent urolithiasis and emphasize the need for systematic aetiological work-up for all patients presenting a first episode of urolithiasis according to the guidelines of the Association Française d'Urologie Stone Committee (CLAFU)...
June 2006: Progrès en Urologie
https://www.readbyqxmd.com/read/16752684/-pancreatic-endocrine-tumors
#13
E Târcoveanu, R Moldovanu, St Georgescu, D Niculescu, C Lupaşcu, G Dimofte
Incidence of the endocrine tumors of the pancreas is about 4 to 10/1.000.000 peoples. We present 10 cases of endocrine pancreatic tumors which were operated in the First Surgical Clinic Iaşi in the last 20 years (1984-2003); these cases represent about 2.21% from all the pancreatic tumors (454 cases). It was 4 insulinoma, 2 gastrinoma, 2 gastrinoma associated with other endocrine neoplasia (Wermer syndrome) and 2 non-functioning endocrine pancreatic tumors. Female/men ratio was 9/1 and median age was about 41...
March 2006: Chirurgia
https://www.readbyqxmd.com/read/16444355/-multiple-endocrine-neoplasia-type-2
#14
REVIEW
Ana Luiza Maia, Jorge Luiz Gross, Marcia Khaled Puñales
The term multiple endocrine neoplasia (MEN) was introduced by Steiner et al. in 1968 to describe disorders that include a combination of endocrine tumors. The Wermer syndrome was designed as MEN 1 and the Sipple syndrome as MEN 2. Sizemore et al. (1974) completed that the MEN 2 category included 2 subgroups: patients with medullary thyroid carcinoma (MTC), pheochromocytoma, and parathyroid disease and a normal appearance (MEN 2A) and other without parathyroid disease but with mucosal neuromas and mesodermal abnormalities (MEN 2B)...
October 2005: Arquivos Brasileiros de Endocrinologia e Metabologia
https://www.readbyqxmd.com/read/15644784/familial-multiple-endocrine-neoplasia-the-first-100-years
#15
REVIEW
J Aidan Carney
In 1903, Erdheim described the case of an acromegalic patient with a pituitary adenoma and three enlarged parathyroid glands. Fifty years later, Underdahl et al reported 8 patients with a syndrome of pituitary, parathyroid, and pancreatic islet adenomas. In 1954, Wermer found that the syndrome was transmitted as a dominant trait. In 1959, Hazard et al described medullary (solid) thyroid carcinoma (MTC), a tumor that later was found to be a component of two endocrine syndromes. The first of these described by Sipple in 1961 comprised pheochromocytoma, MTC, and parathyroid adenoma...
February 2005: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/15170420/-multiple-endocrine-neoplasia-syndroms-type-1
#16
REVIEW
Linas Juodele, Elona Juozaityte, Algimantas Zindzius, Juozas Pundzius
Multiple endocrine neoplasia (MEN) type 1 syndrome or Wermer syndrome is a classical malignant neoplasia syndrome, inherited in the autosomal dominant pattern, when hyperplastic and/or neoplastic injury develops synchronously or metachronously in the cells of the parathyroid gland, pancreas islets, hypophysis, and rarer in other neuroendocrine organs. The syndrome develops when germinative MEN 1--neoplasia suppression gene inactivation mutations occurs. More than 95 percent of patients have this MEN 1 gene mutation, when the penetration of mutation is almost 100 percent...
2004: Medicina
https://www.readbyqxmd.com/read/15067750/-multiple-endocrine-neoplasia-type-1-men1-wermer-syndrome
#17
REVIEW
A Calender, P Goudet et al.
No abstract text is available yet for this article.
November 2003: Annales D'endocrinologie
https://www.readbyqxmd.com/read/15049954/internal-jugular-vein-sampling-in-adrenocorticotropic-hormone-dependent-cushing-s-syndrome-a-comparison-with-inferior-petrosal-sinus-sampling
#18
COMPARATIVE STUDY
Dana Erickson, John Huston, William F Young, Paul C Carpenter, Robert A Wermers, Frank S Bonelli, Claudia C Powell
OBJECTIVE: Distinguishing between pituitary-dependent Cushing's syndrome (CS) and occult ectopic ACTH syndrome can be extremely difficult. Bilateral inferior petrosal sinus sampling has been shown to have the highest diagnostic accuracy in this subtype evaluation. Internal jugular vein sampling (IJVS) has been reported as a potentially safer invasive alternative, but data are limited. Our objective was to compare the sensitivity and specificity of bilateral IJVS and bilateral inferior petrosal sinus sampling (IPSS) in patients with ACTH-dependent CS...
April 2004: Clinical Endocrinology
https://www.readbyqxmd.com/read/14057623/endocrine-adenomatosis-and-peptic-ulcer-in-a-large-kindred-inherited-multiple-tumors-and-mosaic-pleiotropism-in-man
#19
P WERMER
No abstract text is available yet for this article.
August 1963: American Journal of Medicine
https://www.readbyqxmd.com/read/11220895/-multiple-endocrine-neoplasia-of-the-first-type-wermer-s-syndrome
#20
N M Kuzin, A V Egorov, N S Kuznetsov, A N Lotov, M G Lakreeva
11 patients with Wermer's syndrome (WS) aged 24-67 years were treated. They had a total of 30 tumors: 4(13.3%) hypophyseal, 11(36.7%) pancreatic, 9(30%) parathyroid, 4(13.3%) adrenal and 2(6.7%) duodenal. Each patient had two tumors minimum and 8 tumors maximum. In 8 cases WS presented clinically as hypoglycemia, in 3 cases as Zollinger-Ellison syndrome. The diagnosis was made using ultrasonography, CT, MRT and angiography. Cytogenetic examination was made in 2 cases. All the patients were operated. Six of them were operated two times, one--four times...
2000: Klinicheskaia Meditsina
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