Chief Cushie ~MaryO~ Posted April 27, 2007 Chief Cushie Report Share Posted April 27, 2007 http://www.co-endocrinology.com/pt/re/coen...#33;8091!-1 ARTICLE LINKS: Fulltext | PDF (123 K) Adrenocorticotropic hormone-independent Cushing's syndrome. Adrenal cortex Current Opinion in Endocrinology, Diabetes & Obesity. 14(3):219-225, June 2007. Bourdeau, Isabelle; Lampron, Antoine; Costa, Marcia Helena Soares; Tadjine, Mimi; Lacroix, Andre Abstract: Purpose of review: Endogenous Cushing's syndrome is adrenocorticotropic hormone (or corticotropin)-independent in 15-20% of cases. Primary Cushing's syndrome is most often secondary to adrenocortical adenomas or carcinomas, and more rarely to bilateral adrenal hyperplasias. Corticotropin-independent cortisol-producing hyperplasia is caused by micronodular diseases, including primary pigmented nodular adrenocortical disease and nonpigmented micronodular hyperplasia and adrenocorticotropic hormone-independent macronodular adrenal hyperplasia. Primary pigmented nodular adrenocortical disease can be found either alone or in the context of Carney complex, a multiple endocrine neoplasia syndrome. Recent findings: In recent years, the pathophysiology of adrenocortical tumors and hyperplasias became better understood following the identification of genes responsible for syndromes associated with corticotropin-independent Cushing's syndrome and the demonstration of aberrant expression and function of various hormone receptors in adrenocortical adenomas and adrenocorticotropic hormone-independent macronodular adrenal hyperplasia. This article reviews findings on the molecular and genetic aspects of corticotropin-independent Cushing's syndrome including recent gene expression profiling studies of adrenocortical tumors and hyperplasias and animal models that provided clues on the pathogenesis of primary Cushing's syndrome. Summary: A better understanding of molecular mechanisms involved in adrenocortical tumors and hyperplasias may lead to improved diagnostic and prognostic markers and treatment strategies to assist clinicians in the management of corticotropin-independent Cushing's syndrome. ? 2007 Lippincott Williams & Wilkins, Inc. Link to comment Share on other sites More sharing options...
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