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分析1973～1983年间确诊的27例患者。6例于20岁以后方来就诊。临床上突出表现为男性化征象,儿童期生长过速,骨骺早期愈合,13岁以后多停止生长。实验室检查:尿17—KS,尿17—KGS均显著增高,并能被中剂量地塞米松抑制试验降为正常,性染色体或染色质检查均符合女性。长期氢皮质素治疗使尿17—KS降为正常是本病最基本的疗法。早期诊断,及时持续激素治疗,可使Prader Ⅰ、Ⅱ型部分患者接近正常免于手术。Ⅲ—Ⅴ型患者须在激素治疗病情稳定后,方可手术。近年来,作者改进手术方法,将阴蒂全切改为大部分切除,保留龟头和完整的阴茎背动、静脉及神经,可能有益性生活。本组长期随防观察17例,显效10例,良效5例,效差2例。疗效与初治年龄密切相关,治疗愈早。效果愈好。Prader Ⅰ、Ⅱ型疗效较好。原发闭经疗效差。
27 patients diagnosed between 1973 and 1983 were analyzed. Six cases were treated after the age of 20. Outstanding clinical manifestations of masculine signs of childhood over-growth, epiphyseal early healing, more than 13 years of age to stop growing. Laboratory tests: urinary 17-KS, urinary 17-KGS were significantly higher, and can be moderate dexamethasone suppression test reduced to normal, sex chromosomes or chromatin examination are in line with women. Long-term hydrogen cortisol treatment of urinary 17-KS reduced to normal is the most basic therapy of this disease. Early diagnosis, timely and continuous hormone therapy, Prader Ⅰ, Ⅱ type of part of the patient close to normal from surgery. Ⅲ-Ⅴ type of patients to be stable in hormone therapy before surgery. In recent years, the author has improved the surgical method, changing the clitoris to the majority of resections, preserving the glans penis and intact penis dorsal, veins and nerves, which may be beneficial to life. The long-term prevention and treatment of this group with 17 cases, markedly effective in 10 cases, good effect in 5 cases, effective in 2 cases. The curative effect is closely related with the initial treatment age, the earlier the treatment. The effect is better. Prader Ⅰ, Ⅱ type effect is better. Poor primary amenorrhea.