先天性肾上腺皮质增生症是一组肾上腺皮质激素生物合成酶系中某一或几种酶的先天性缺陷,而使皮质醇等激素水平改变所致的一组疾病。最常见的酶缺陷为21-羟化酶缺陷,其次为11β-羟化酶缺陷。17α-羟化酶缺陷非常罕见。本科2010年7月收治1例,现报告如下。 Congenital adrenal hyperplasia is a group of adrenal cortical hormone biosynthesis enzymes in one or a few congenital defects in enzymes, leaving cortisol and other hormones caused by a change in the group of diseases. The most common enzyme defects were 21-hydroxylase deficiency, followed by 11β-hydroxylase deficiency. 17α-hydroxylase deficiency is very rare. Bachelor in July 2010 admitted in 1 case, are as follows.