lipoid adrenal hyperplasia

lipoid congenital adrenal hyperplasia the most severe type of congenital adrenal hyperplasia, caused by mutation in either of two genes (STAR, locus: 8p11.2, or CYP11A, locus: 15q23-q24) that encode proteins involved in steroidogenesis. It is characterized by accumulation of lipids and cholesterol in the adrenal cortex, decreased or absent steroid hormones, severe salt wasting, sexual infantilism, and male pseudohermaphroditism; the adrenal insufficiency is usually lethal. See table at congenital adrenal hyperplasia.