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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。
In this paper, 31 cases of congenital adrenal hyperplasia (CAH) with prednisone and external genital deformity correction surgery. External genital abnormalities according to Prader classification, including type Ⅰ in 5 cases, type Ⅱ in 9 cases, type Ⅲ in 17 cases. In addition to 1 case of amenorrhea, the rest are primary amenorrhea. All cases of breast were not developed, were male nipples before treatment for dexamethasone inhibition test, after the test 17-KS values were significantly decreased, according to the pre-test 17-KS value of prednisone dosage. All patients have improved secondary sexual characteristics, menstrual cramps. Among them, 11 were married, 6 were pregnant, and 4 were full-term pregnant women and children were healthy. Observation found that some patients still maintain the menstrual cycle after withdrawal. This article on the CAH patients need to take the issue of life-long medication were discussed.