hrp0086p2-p50 | Adrenal P2 | ESPE2016
Kopylova Irina
, Orlova Elizaveta
, Yarovaya Irina
, Kareva Maria
, Peterkova Valentina
Background: Congenital lipoid adrenal hyperplasia (CLAH) is the most severe form of congenital adrenal hyperplasia, characterized by lack of synthesis of all kinds of steroids in adrenals and gonads due to defects in gene of Steroidogenic Acute Regulatory protein (StAR). 46,XX patients can have a spontaneous puberty due to residual estrogen synthesis by a StAR-independent pathway in ovary. Development of ovarian cysts may be derived from persistent anovulation and impairment o...