先天性肾上腺皮质增生症31例疗效观察

Clinical observation on congenital adrenocortical hyperplasia:Analysis of 31 cases.

本文对31例先天性肾上腺皮质增生症(CAH)采用强的松及外生殖器畸形矫正手术。外生殖器异常按 Prader 的分型,其中Ⅰ型5例,Ⅱ型9例,Ⅲ型17例。除1例无闭经外,其余均为原发性闭经。所有病例乳房均未发育,呈男性乳头,治疗前先作地塞米松抑制试验,试验后17—KS 值均明显下降,根据试验前17—KS 值选择强的松用量。全部患者第二性征得到改善,月经来潮。其中已婚11例,6例妊娠,4例足月妊娠,母婴健康。观察中发现部分病人停药后仍能维持月经周期。本文就此对 CAH 病人是否需终生服药问题进行了讨论。

Thirty patients with congenital adrenocortical hyper- plasia(CAH)were treated with oral prednisoione and external genital organ orthomorphia.According to Pracler's classification of the abnormality of the exter- nal genital organs,5 cases were of type Ⅰ.,9 were of type Ⅱ,and the rest 17 were of type Ⅲ.All of the t patients had primary amenorrhea except for one whose mensis was normal.Breast were not well de- veloped resembling those of males.Dexamethasone in- hibition tests were performed before the treatment, and doses of prednisolone were given according to the urinary levelof 17-ketosteroid before the test.Sec- ond sexual signs were improved and menstruations oc- curred in all the patients after the treatment.Eleven of them were married.Six became pregnant.Four had term deliveries with healthy babies.Some of the pa- tients still maintained normal menstrual cycles after the cessation of the medication.The paper also dis- cussed whether CAH patients needed life long medica- tion.