雄激素不敏感综合征临床诊治分析

发表时间：2014-07-08 浏览次数：1184次

引用：

王周，撒应龙，叶绪晓等.雄激素不敏感综合征临床诊治分析[J].临床泌尿外科杂志.2013,28(4):287-291

关键词：

雄激素受体；睾丸；雄激素不敏感综合征；

作者：

王周，撒应龙，叶绪晓，张炯，徐月敏

作者单位：

苏州大学医学部；上海交通大学附属第六人民医院泌尿外科；

出版年份：

2013

期刊页数：

287-291

收录者：

知网

摘要：

目的:探讨雄激素不敏感综合征(androgen insensitivity syndrome,AIS)患者临床诊断和治疗方法,提高对AIS的诊治效率。方法:回顾性分析我院1985年9月～2012年6月收治6例AIS患者的临床特征,诊断与处理。所有患者均进行了染色体,性激素六项,下腹部B超,术中切除组织病理检测等检查,其中5例患者给予手术切除性腺组织,术后给予小剂量雌激素治疗,1例给予保守治疗。结果:AIS患者多以原发闭经就诊,染色体核型均为46,XY。性激素六项中除了睾酮和LH升高外,其余项未见明显异常。术中切除组织病理检查提示睾丸间质细胞和支持细胞组成的正常睾丸组织,未发现睾丸肿瘤。所有手术患者均未出现女性第二性征发育停滞及骨质疏松等性腺切除后并发症。结论:AIS患者多以"原发闭经就诊",染色体核型分析联合下腹部B超检查均能有助于AIS快速检出,手术切除性腺具有安全行和可行性。同时加强AIS遗传病史家族胎儿染色体筛查有重要意义。 Objective:Obiective To工nvestigate the diagnosis and the treatment of androgen insensitivity syn- drome patients to improve the efficiency. Method; A retrospective analysis were performed with total of six cases of androgen insensitivity syndrome patients on clinical features。diagno、上、and treatment from September 1985 to June 2012 in our hospital. A11 surgical patients have examined the chromosome, the Sex hormone levels, the lower ab- dominal B ultrasound .the pathological examination and other tests. Five cases of these were treated with surgical way to removal the gonadal tissue and were given the long-term treatment with low-dose estrogen after the opera- tion. One case have taken the conservative treatment. Result:Androgen 'insensitivity syndrome patients often go to treat as the reason of primary amenorrhea, These people often have:karyotype of 46，XY and have a normal hor- mone levels except testosterone and LH's levels increased. Intraoperative removal of pathological examinations show a normal testicular tissue which are composed of Leydig cells and Sertoli cells，and have not found any testic- ular cancer. A11 operations have not appeared the postoperative complications which like female secondary sexual characteristics stagnation and osteoporosis. Conclusion: Androgen insensitivity syndrome patients often go to treat as the reason of primary amenorrhea. The karyotype analysis and the lower abdominal B ultrasound test can con- tribute to diagnose the androgen insensitivity syndrome rapidly, It is a safe and reliable way to removal the gonads of these patients. It have a Significance to Strengthen the chromosome screening in Androgen insensitivity syn- drome patientsfamily.

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