11-?-Hydroxylase deficiency

Abstract

Congenital adrenal hyperplasia (CAH) due to 11-?-Hydroxylase deficiency has been reported to be 15% of all CAHs in some ethnic groups of middle east countries. During the last 10 years we found 58 cases of CAH in the children's medical center in Tehran. 6 of these had 11-?-Hydroxylase deficiency. We report on these cases and give a brief review of clinical aspects and pathophysiology of this congenital anomaly.