摘要
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。
Clinical manifestations of two groups of 21-hy-
droxylase deficiency(21HD) were compared in this
report.Group 1 consisted of 8 female cases of late-on-
set form and group 2 consisted of 42 cases of classical
form,without salt-loosing.The results were:(1) In
group 1,6 had spontaneous menstrual cycles and 5
had varying degrees of breast development(Tanner
stage Ⅲ～Ⅳ) before treatment.While all cases in
group 2 showed primary amenorrbea and no breast
development,except in one with Tanner stage Ⅱ.(2)
Virilization in group 1 was much milder than in group
2.All cases in group 1 had mild clitoromegaly with-
out labial fusion.While in group 220 cases had defi-
nite labial fusion.In group 1 only 4 cases had some
hirsutism,menstrual irregularity and infertility.No
definite male body built,adam apple,low voice or
beard.While in group 225 cases of above I0 years
old had severe degrees of virilization.
出处
《生殖医学杂志》
CAS
1992年第2期92-94,共3页
Journal of Reproductive Medicine
