Possible association between Carney complex and multiple endocrine neoplasia type 1 phenotypes
نویسندگان
چکیده
منابع مشابه
Possible association between Carney complex and multiple endocrine neoplasia type 1 phenotypes.
Carney Complex (CNC) and Multiple Endocrine Neoplasia type 1 (MEN1) are forms of multiple endocrine neoplasia of dominant autosomal inheritance. Diagnosis of CNC occurs when two major criteria (lentiginoses, primary pigmented nodular adrenocortical disease, cardiac and cutaneous myxomas, acromegaly, testicular neoplasias, thyroid cancer) are observed and/or a major criterion associated with a s...
متن کاملMultiple endocrine neoplasia type 1
Combined clinical and laboratory investigations of MEN-1 have resulted in an increased understanding of this disorder, which may be inherited as an autosomal dominant condition. Defining the features of each disease manifestation in MEN-1 has improved patient management and treatment and has facilitated a screening protocol. Application of the techniques of molecular biology has enabled the ide...
متن کاملMultiple endocrine neoplasia type 1.
Multiple endocrine neoplasia-1 (MEN-1) is an autosomal dominant inherited syndrome that occurs due to inactivating mutations of the MEN1 gene locus, coding for a tumor-suppressor protein, menin. The components of MEN-1 are hyperparathyroidism due to multiple parathyroid adenomas, pancreatic neuroendocrine tumors, and pituitary adenomas, in addition to some less common neoplastic manifestations....
متن کاملDiscovery of the Carney Complex, a Familial Lentiginosis-Multiple Endocrine Neoplasia Syndrome: A Medical Odyssey
In 1981, study of the adrenal pathology in four cases of Cushing syndrome led to characterization of a unique disorder termed primary pigmented nodular adrenal disease (PPNAD). Review of the literature showed that the condition occurred in two families. In one, it had affected two siblings; a third sibling who did not have Cushing syndrome died of cardiac myxoma. To test the hypothesis that the...
متن کاملMultiple endocrine neoplasia type 1 (MEN1) and type 4 (MEN4)
Multiple endocrine neoplasia (MEN) is characterized by the occurrence of tumors involving two or more endocrine glands within a single patient. Four major forms of MEN, which are autosomal dominant disorders, are recognized and referred to as: MEN type 1 (MEN1), due to menin mutations; MEN2 (previously MEN2A) due to mutations of a tyrosine kinase receptor encoded by the rearranged during transf...
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ژورنال
عنوان ژورنال: Arquivos Brasileiros de Endocrinologia & Metabologia
سال: 2008
ISSN: 0004-2730
DOI: 10.1590/s0004-27302008000800025