Congenital Adrenal Hyperplasia

This disorder, also called adrenogenital syndrome
(AGS, McKusick 201910), is caused by a
genetically determined deficiency of cortisol, a
steroid hormone produced in the fetal adrenal
cortex. A compensatory increase in adrenocortical
hormone (ACTH) excretion leads to secondary
enlargement (hyperplasia) of the
adrenal cortex (congenital adrenal hyperplasia),
increased production of prenatal
steroids and their metabolites with androgenic
effects, and incomplete female sex differentiation.