性发育异常的临床分型及治疗  

Clinical classification and treatment of sexual abnormalities

作　　者：唐艳[1,2] 乔林[1] 李美胜[3] 徐克惠[1] 

机构地区：[1]四川大学华西附属第二医院,成都610041 [2]四川省都江堰医疗中心 [3]四川大学华西医院

出　　处：《山东医药》2010年第26期16-18,共3页Shandong Medical Journal

摘　　要：目的探讨性发育异常的临床分型及治疗方法。方法对61例性发育异常患者的临床表现、生殖器官发育情况、血清性激素水平、染色体核型等进行分析,按照中华妇产科学(2版)新的分型方法分型并给予相应治疗。结果 61例中,诊断为Turner综合征7例、XO/XY性腺发育不全5例、超雌综合征1例、XX单纯性腺发育不全6例、XY单纯性腺发育不全16例、真两性畸形2例、睾丸退化综合征1例、21-羟化酶缺乏7例、17α-羟化酶缺乏1例、雄激素不敏感综合征12例、原发性闭经伴常染色体异常3例。35例行双侧性腺切除术,病理检查发现性腺恶性肿瘤5例;同时结合患者社会性别、骨龄等给予相应药物治疗。结论性发育异常的临床分型比较复杂,确诊后根据临床分型及社会性别不同可采用手术及激素疗法。Objective To study the clinical classification and therapy methods of sexual abnormality.Methods 61 patients with sexual abnormality were analized retrospectively with clinic feature,development of genital organs,sex hormones and karyotype.Results 61 cases included 7 cases of Turner syndrome,5 cases of XO/XY gonadal dysgenesis,1 case of superfemale,6 cases of XX pure gonadal dygenesis,16 cases of XY pure gonadal dygenesis,2 cases of true hermaphroditism,1 case of testicle degneration syndrome,7 cases of 21-Hydroxylase Deficiency,1 case of 17a-Hydroxylase deficiency,12 cases of androgen insensitivity syndrome,3 cases of Primary amenorrhea with autosomal abnormalities.Both of the gonad of 35 cases were resected.5 cases with malignant tumor were diagnosed by pathological examination.The patients were treated with medication according to the diagnosis,social gender and bone age.Conclusion The clinical classification of sexual abnormality is complicated.Surgery and hormone therapy are the main therapy methods.

关 键 词：性发育异常 两性畸形 性腺 性激素 染色体 

分 类 号：R596.1[医药卫生—内科学]