نتایج جستجو برای: men2a

تعداد نتایج: 147  

Journal: :Journal of the American College of Surgeons 2004
Linwah Yip Jeffrey E Lee Suzanne E Shapiro Steven G Waguespack Steven I Sherman Ana O Hoff Robert F Gagel James F Arens Douglas B Evans

BACKGROUND Surgical treatment of hereditary pheochromocytoma remains controversial because of the need for lifelong corticosteroid therapy and the risk of Addisonian crisis associated with bilateral total adrenalectomy. We examined our large series of patients with hereditary pheochromocytoma to evaluate postsurgical outcomes, particularly in those who underwent cortical-sparing adrenalectomy. ...

2011
Xiao-Ping Qi Ju-Ming Ma Zhen-Fang Du Rong-Biao Ying Jun Fei Hang-Yang Jin Jian-Shan Han Jin-Quan Wang Xiao-Ling Chen Chun-Yue Chen Wen-Ting Liu Jia-Jun Lu Jian-Guo Zhang Xian-Ning Zhang

BACKGROUND Whole exome sequencing provides a labor-saving and direct means of genetic diagnosis of hereditary disorders in which the pathogenic gene harbors a large cohort of exons. We set out to demonstrate a suitable example of genetic diagnosis of MEN 2A/FMTC (multiple endocrine neoplasia type 2/familial medullary thyroid carcinoma) using this approach. METHODOLOGY/PRINCIPAL FINDINGS We se...

Journal: :Journal of clinical research in pediatric endocrinology 2016
Berna İmge Aydoğan Bağdagül Yüksel Mazhar Müslüm Tuna Mehtap Navdar Başaran Ayşen Akkurt Kocaeli Melek Eda Ertörer Kadriye Aydın Sibel Güldiken Yasin Şimşek Züleyha Cihan Karaca Merve Yılmaz Müjde Aktürk İnan Anaforoğlu Nur Kebapçı Cevdet Duran Abdullah Taşlıpınar Mustafa Kulaksızoğlu Alptekin Gürsoy Selçuk Dağdelen Murat Faik Erdoğan

OBJECTIVE This retrospective multicenter study, centrally conducted and supported by the Society of Endocrinology and Metabolism of Turkey, aimed to evaluate the impact of free RET proto-oncogene testing in medullary thyroid carcinoma (MTC) patients. Surgical timing, adequacy of the treatment, and frequency of prophylactic thyroidectomy (PTx) in mutation carriers were also assessed. METHODS G...

Journal: :Hormones 2007
Sotirios Bethanis George Koutsodontis Theodosia Palouka Christos Avgoustis Drakoulis Yannoukakos Thalia Bei Savas Papadopoulos Dimitrios Linos Stylianos Tsagarakis

Multiple endocrine neoplasia type 2A (MEN2A) is a syndrome of familial neoplasias characterized by medullary thyroid carcinoma (MTC), pheochromocytoma and hyperplasia of the parathyroid glands. RET protooncogene mutations are responsible for MEN 2A. Mutations in exons 10 or 11 have been identified in more than 96% of patients with MEN 2A. We herein report for the first time a patient with MEN 2...

Journal: :European journal of endocrinology 2008
L Foppiani F Forzano I Ceccherini W Bruno P Ghiorzo F Caroli P Quilici R Bandelloni A Arlandini G Sartini M Cabria P Del Monte

INTRODUCTION Calcitonin measurement is advised in the diagnosis of thyroid nodules, as it is an accurate marker of medullary thyroid carcinoma (MTC). C-cell hyperplasia (CCH)-induced hypercalcitoninemia cannot be distinguished from that induced by MTC, unless surgery is performed. CASE We report the clinical and biological features of a patient with a family history of cancer, including melan...

دکتر فریدون عزیزی, , دکتر ایرج نبی‌پور, , دکتر مهدی هدایتی, , دکتر نصراله رضایی قلعه, ,

مقدمه: سرطان مدولری تیروئید (MTC) به دو نوع اسپورادیک و ارثی بروز می‌کند. ژن عامل حساسیت به فرم ارثی MTC، پروتوانکوژن رت (RET) است. هدف از این مطالعه، ارزیابی شیوع جهش‌های ژرم لاین رت در اگزون‌های 10 و 11 در میان مبتلایان به این بیماری در جمعیت ایرانی است. مواد و روش‌ها: 57 بیمار غیر منسوب، مبتلا به MTC در این مطالعه مورد بررسی قرار گرفتند که میانگین سنی آنها 0/40 سال (با انحراف معیار 5/11 سال)...

Journal: :Cancer research 2005
Ivan Plaza Menacho Roelof Koster Almer M van der Sloot Wim J Quax Jan Osinga Tineke van der Sluis Harry Hollema Grzegorz M Burzynski Oliver Gimm Charles H C M Buys Bart J L Eggen Robert M W Hofstra

The RET proto-oncogene encodes a receptor tyrosine kinase whose dysfunction plays a crucial role in the development of several neural crest disorders. Distinct activating RET mutations cause multiple endocrine neoplasia type 2A (MEN2A), type 2B (MEN2B), and familial medullary thyroid carcinoma (FMTC). Despite clear correlations between the mutations found in these cancer syndromes and their phe...

2017
Xiwei Zhang Dangui Yan Junyi Wang Hanfeng Wan Yongxia Zhang Yabing Zhang Yuqin He Wensheng Liu Bin Zhang

OBJECTIVE The American Thyroid Association (ATA) proposed a new risk classification for hereditary medullary thyroid carcinoma (MTC) in 2015. This study aimed to assess whether the new guidelines are suitable for the Chinese population, and reported our experience on prophylactic thyroidectomy. METHODS A total of 73 patients from 22 families were screened as rearranged during transfection (RE...

Safura Pakizehkar, Samaneh Hosseinzadeh,

Medullary thyroid cancer accounts for 5-10% of thyroid carcinomas. RET proto-oncogene mutations occur in all of the hereditary MTCs and about 66% of the sporadic MTCs. So, the detection of the RET mutations is necessary for rapid and proper diagnosis and treatment. This systematic review seeks to find a comprehensive list of RET gene mutations in the diagnosis of medullary thyroid cancer. The ...

2014
Stephen C. Kales Marion M. Nau Anand S. Merchant Stanley Lipkowitz

The Cbl proteins (Cbl, Cbl-b, and Cbl-c) are a highly conserved family of RING finger ubiquitin ligases (E3s) that function as negative regulators of tyrosine kinases in a wide variety of signal transduction pathways. In this study, we identify a new Cbl-c interacting protein, Enigma (PDLIM7). This interaction is specific to Cbl-c as Enigma fails to bind either of its closely related homologues...

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