真两性畸形2例报告

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目的:总结对真两性畸形的诊治经验。方法:回顾性分析2 例本病患者的临床资料。结果:对1 例社会性别为女性,染色体为46,XX,内分泌检查证明卵巢有功能者行阴茎海绵体及双侧隐睾切除,阴蒂阴道成形术,术后小剂量雌激素治疗14 个月。随访2 年,有规则月经,第二性征向女性方向发展,内分泌功能基本正常。另1 例社会性别为男性,染色体为46,XX/46,XY,男性性征明显,内分泌功能为男性者,行隐睾下降固定及Ⅱ期尿道成形术,并切除发育不全的输卵管及卵巢。术后生长发育与同龄男孩无差异,阴茎和睾丸大小及形态基本正常。结论:治疗本病应根据患者性腺功能情况和外生殖器矫形的可能性确定性别而行相应的外生殖器矫形术,切除与确定性别相抵触的性腺,术后或青春期可酌情补充性激素 Objective: To summarize the diagnosis and treatment of true hermaphroditism. Methods: The clinical data of 2 patients with this disease were retrospectively analyzed. Results: One case of gender female, chromosome 46, XX, endocrine examination showed ovarian function in patients with penile cavernous and bilateral excision of the cryptorchidism, clitoris vaginoplasty, postoperative low-dose estrogen therapy for 14 months. Follow-up 2 years, regular menstruation, secondary sexual orientation to the female direction, endocrine function was normal. Another case of gender male, chromosome 46, XX / 46, XY, obvious male sexual characteristics, endocrine function of men, line reduction of cryptorchidism and stage Ⅱ urethroplasty, and removal of hypoplastic oviducts and ovaries. Postoperative growth and development no difference with the same age boys, penis and testicular size and shape was normal. Conclusion: The treatment of this disease should be based on the patient’s gonadal function and genital Orthopedic possibility to determine the gender and the line of external genital Orthopedic surgery, resection and gender-specific gonad excision, postoperative or adolescent may be appropriate to add sex hormones
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