Bilateral Adrenal Cushing's Syndrome: Macronodular Adrenal Hyperplasia and Primary Pigmented Nodular Adrenocortical Disease
作者： André Lacroix , Isabelle Bourdeau
DOI: 10.1016/J.ECL.2005.01.004
关键词:
摘要: Corticotropin (ACTH)-independent bilateral macronodular adrenal hyperplasia (AIMAH) and primary pigmented nodular adrenocortical disease (PPNAD) are responsible for approximately 10% of Cushing's syndrome. AIMAH also can be present as subclinical incidentalomas in sporadic or familial forms. Diverse aberrant hormone receptors have been found to implicated the regulation steroidogenesis pathophysiology AIMAH. PPNAD alone context Carney complex, a multiple endocrine neoplasia Additionally, it secondary mutations type 1 alpha-regulatory subunit cAMP-dependent protein kinase A (PRKARIA). Strategies investigation treatment discussed.
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