Which condition is associated with Congenital adrenal hypoplasia?: (A) Male pseudohermaphroditism, (B) Female pseudohermaphroditism, (C) True pseudohermaphroditism, (D) Sequential pseudohermaphroditism
Answer is A. * X-linked congenital adrenal hypoplasia (AHC) is a rare developmental disorder of the human adrenal coex and is caused by deletion or mutation of the DAX-1 gene. * DAX1 necessary for differentiation of the definitive adult adrenal coex * Male pseudohermaphroditism results from inadequate androgen secretion or inappropriate androgen action Reference: GHAI Essential pediatrics, 8th edition