선천성 부신증식증환자의 치험 1례

Abstract

Congenital adrenal hyperplasia(CAH) is an autosomal recessive disorder caused by a deficiency of one or more enzymes necessary for the synthesis of cortisol. The most common and best known form of CAH is 21-hydroxylase deficiency. In CAH, the major clinical manifestations are due to an increased ACTH output and an excessive secretion of androgen. A case of female pseudohermaphroditism due to congenital adrenal hyperplasia is presented with a brief review of literatures.