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Wermer syndrom

Imanda M E Alons, Ben F J Goudsmit, Korné Jellema, Marianne A A van Walderveen, Marieke J H Wermer, Ale Algra
OBJECTIVES: Patients with acute headache increasingly undergo CT-angiography (CTA) to evaluate underlying vascular causes. The aim of this study is to determine clinical and non-contrast CT (NCCT) criteria to select patients who might benefit from CTA. METHODS: We retrospectively included patients with acute headache who presented to the emergency department of an academic medical center and large regional teaching hospital and underwent NCCT and CTA. We identified factors that increased the probability of finding a vascular abnormality on CTA, performed multivariable regression analyses and determined discrimination with the c-statistic...
May 9, 2018: Brain and Behavior
Imanda M E Alons, Ben F J Goudsmit, Korne Jellema, Marianne A A van Walderveen, Marieke J H Wermer, Ale Algra
BACKGROUND: Patients with acute severe headache, normal neurological examination, and a normal noncontrast head computed tomography (NCCT) may still have subarachnoid hemorrhage, cerebral venous thrombosis (CVT), cervical arterial dissection, or reversible cerebral vasoconstriction syndrome (RCVS). Computed tomography angiography (CTA) is used increasingly in the emergency department for evaluating this, but its added value remains controversial. METHODS: We retrospectively collected data on the diagnostic yield of CTA in patients with acute severe headache, normal neurological examination, and normal NCCT who received additional CTA in the acute phase in 2 secondary referral centers for vascular neurology...
April 2018: Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association
Haikun Liu, Dongsheng Guo, Aynisahan Ruzi, Yan Chen, Tingcai Pan, Fan Yang, Jialiang Li, Kecheng Xu, Tiancheng Zhou, Dajiang Qin, Yin-Xiong Li
Human induced pluripotent stem cells (hiPSCs) may provide potential resource for regenerative medicine research, including generation of insulin-producing cells for diabetes research and insulin production. Testosterone (T) is an androgen hormone which promotes protein synthesis and improves the management of type 2 diabetes in clinical studies. Concurrently, co-existed hyperandrogenism and hyperinsulinism is frequently observed in polycystic ovary syndrome, congenital adrenal hyperplasia and some of Wermer's syndrome...
2017: PloS One
Halil Korkut Daglar, Ayse Kirbas, Ebru Biberoglu, Bergen Laleli, Nuri Danisman
Multiple Endocrine Neoplasia Type 1 (MEN1) or Wermer's syndrome is a rare hereditary endocrine syndrome with high penetrance caused by mutations in MEN1 tumor suppressor gene. MEN1 is characterized by hyperplasia or tumoral enlargement in a number of endocrine organs (parathyroid glands, pancreas, pituitary gland, adrenal gland) and it could be hormonally active or inactive. MEN1 is a significant cause of morbidity due to hormone secretion and mass effect. Since it is a rare condition, there are no guidelines with respect to the follow-up of pregnant women with MEN1...
July 2016: Journal of Experimental Therapeutics & Oncology
Keyur Desai, Joshua D Wermers, Nebiyu Beteselassie
Lemmel syndrome occurs when a duodenal diverticulum causes obstructive jaundice due to a mechanical obstruction of the common bile duct. Additional pathophysiologic processes may also contribute to the development of Lemmel syndrome. These include duodenal diverticula causing dysfunction of the sphincter of Oddi as well as compression of the common bile duct by duodenal diverticula. It is uncommon for duodenal diverticulum to become inflamed. We report the case of a 25-year-old female presenting with unintentional weight loss and fatigue...
March 1, 2017: Curēus
M S Ekker, M J H Wermer, N P Riksen, C J M Klijn, F E de Leeuw
- In virtually all age groups, the incidence of ischemic stroke is higher in men. However, in women aged between 25-49 years the prevalence is higher than in men. Female-specific risk factors and disorders may explain this peak.- Pregnancy and the post-partum period are associated with physiological changes which can increase blood coagulation. Complications during pregnancy may also increase the risk of ischaemic stroke.- Migraine with aura and antiphospholipid syndrome are disorders which are also associated with an increased risk of ischaemic stroke...
2016: Nederlands Tijdschrift Voor Geneeskunde
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...
December 0: Vnitr̆ní Lékar̆ství
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
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
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
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
Timothy L Frankel, Paul G Gauger
No abstract text is available yet for this article.
April 2009: Archives of Surgery
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
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
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
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
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
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
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
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
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