先天性肾上腺皮质增生症（ＣＡＨ）属于肾上腺类固醇激素合成障碍的遗传性疾病。本症由于遗传缺陷致类固醇激素合成途径中一些必需酶缺乏而引起。其中２１羟化酶缺乏占ＣＡＨ的９０％以上，本文２１例均为２１羟化酶完全缺乏和部分缺乏，两者均表现为男性化和男性化伴失盐... Congenital adrenal hyperplasia (CAH) belongs to the genetic disorder of adrenal steroid hormone synthesis. The disease due to genetic defects in steroid hormone synthesis pathway caused by some necessary enzyme deficiency. Which 21 hydroxylase deficiency accounts for more than 90% of CAH, 21 cases of this article are 21 cases of complete lack of hydroxylase and part of the lack of both male and masculine with loss of salt ...