hrp0097p1-402 | Adrenals and HPA Axis | ESPE2023

Micronodular bilateral adrenal hyperplasia: about 2 cases in early childhood

Lambert Sophie , A Stratakis Constantin , Chivu Olimpia , Boros Emese , Heinrichs Claudine , Ulgiati Fiorenza , Brachet Cécile

Background: Micronodular bilateral adrenal hyperplasia (MiBAH) is a rare cause of adrenal Cushing syndrome (CS) that may be subdivided in two main entities: primary pigmented nodular adrenocortical disease (PPNAD) and isolated micronodular adrenocortical disease (i-MAD). The most common presentation of MiBAH is familial PPNAD as part of Carney complex (CNC). The underlying genetic defect in most forms of PPNAD is inactivating germline mutations of the PRKAR1A ...