本文对8例迟发型和42例非失盐性经典型21-羟化酶缺陷症(21 H D)患者的临床表现进行了比较。两组患者全部为女性。结果发现:(1)迟发型患者女性特征有较好发育,8例中6例月经已来潮,4例基本正常,5例有不同程度乳房发育;经典型全部为原发性闭经,仅1例乳房为Ⅱ期,余全部为Ⅰ期;(2)迟发型患者男性化程度轻,虽全部有轻度阴蒂肥大,但均无明显大阴唇融合;经典型阴蒂肥大重,且42例中有20例大阴唇融合显著。迟发型8例中有4例仅有多毛及月经紊乱或不育,无明显男性体型、喉结增大、嗓音增粗及胡须;经典型10岁以上的25例男性化程度严重。 This article compared the clinical presentation of 8 patients with late-onset and 42 non-loss-of-life with classic 21-hydroxylase deficiency (21 H D). Both groups were women. The results showed that: (1) The characteristics of women with late-onset type were well developed. Among the 8 cases, 6 cases of menstruation had been waved, 4 cases were normal and 5 cases had different degrees of breast development. The classic type was all primary amenorrhea and only 1 case Breast Ⅱ, more than all Ⅰ; (2) late-onset patients with less degree of masculinity, although all mild clitoral hypertrophy, but no significant fusion of the labia majora; classic clitoris hypertrophy, and 42 cases of 20 Cases of labia majora fusion significantly. There were only 4 hirsutism and menstrual disorders or infertility in 8 cases of delayed type, no obvious body size, enlarged Adam’s apple, thickened voice and whiskers; 25 cases of classic over 10 years old were highly masculine.