Genetics of primary macronodular adrenal hyperplasia.

摘要: ACTH-independent macronodular adrenal hyperplasia is a rare cause of Cushing's syndrome (CS), accounting for <2% all endogenous CS cases; however it more frequently identified incidentally with sub-clinical cortisol secretion. Recently, secretion has been shown to be regulated by ectopic corticotropin, which in turn produced clusters steroidogenic cells the hyperplastic nodules. Hence, term 'ACTH-independent' not entirely appropriate this disorder. Accordingly, disease designated primary (PMAH) review article. The means production PMAH despite suppressed levels ACTH pituitary origin exceedingly complex. Several molecular events have proposed explain enhanced secretion, increased cell proliferation, and nodule formation PMAH. Nonetheless, precise sequence mechanisms underlying condition remain unclear. purpose therefore present new insights on genetic profile pathophysiology, discuss implications progression.