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为了探讨男性 17α-羟化酶缺乏所致两性畸形的诊断方法及治疗效果。总结了我院 1987年 7月～ 1997年 3月共收治 7例确诊为 17α-羟化酶缺乏患者 ,采取双侧性腺切除术加糖皮质激素及雌激素替代治疗。所有患者均维持原女性社会性别及外貌 ,2例已婚者性生活满意。认为 17α-羟化酶缺乏所致男性假两性畸形 ,虽染色体核型为 4 6 ,XY,但外生殖器为女性 ,难以恢复男性应有的性功能 ,发育不良的睾丸组织有潜在恶变可能。因此 ,维持女性社会性别 ,切除两侧性腺 ,婚前行阴道扩张或成形术治疗是最佳选择
In order to explore male 17α-hydroxylase deficiency caused by diagnosis and treatment of amphipods. Summary of our hospital from July 1987 to March 1997 were treated in 7 cases were diagnosed as 17α-hydroxylase deficiency in patients with bilateral gonadal resection plus glucocorticoid and estrogen replacement therapy. All patients maintained the original female gender and appearance, two married people were satisfied with their sex life. It is considered that 17α-hydroxylase deficiency causes males’ bisexual deformity. Although the chromosome karyotype is 46, XY, the external genitalia is female, it is difficult to restore the sexual function that should be possessed by the male. The dysplastic testes may have potential malignant transformation. Therefore, to maintain female gender, removal of both sides of the gonads, vaginal pre-marriage or plastic surgery is the best choice