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雄激素不敏感综合征临床诊断及治疗:附七例病例分析 被引量:1

Clinical Diagnosis and Treatment of Androgen Insensitivity Syndrome: Analysis of Seven Cases
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摘要 目的分析雄激素不敏感综合征(AIS)的诊断及治疗特点。方法回顾性分析首都医科大学附属北京妇产医院1997—2012年收治的7例AIS患者的临床资料,对疾病的诊断及治疗进行总结。结果 7例患者外观均呈不同程度女性型,染色体核型为46,XY,5例患者睾酮水平高于正常男性。7例均行手术切除睾丸,5例合并睾丸肿瘤,术后均口服雌激素替代治疗,无性别认知障碍。结论原发闭经患者睾酮为男性水平、染色体核型为46,XY时应考虑为AIS,青春期前的患者应行人绒毛膜促性腺激素(HCG)试验了解性腺功能。根据AIS分类选择手术时机及手术方式可以提高患者生活质量、减少肿瘤发生。 Objective To analyze the characteristics of the diagnosis and treatment of androgen insensitivity syndrome( AIS). Methods We made a retrospective analysis of the clinical data of 7 cases of AIS who were admitted into 1997 to 2012 in Beijing Obstetrics and Gynecology Hospital,Capital Medical University from 1997 to 2012,and we summarized the diagnosis and treatment of the disease. Results Female appearance of different degrees appeared in 7 patients with a karyotype of 46 and XY,and 5 patients had higher testosterone level than normal males. There were 7 patients who had surgical removal of testicle and 5patients who also had testiculoma,and these patients all received oral administration of estrogen as the replacement therapy after surgery and had no gender identity disorder. Conclusion For primary amenorrhea patients whose testosterone level is within the male range and a karyotype of 46 and XY,AIS should be considered. For preadolescent patients,HCG test should be conducted to evaluate the sex gland function. Choosing timing and methods of surgery according to the AIS types can improve the quality of life of patients and reduce tumorigenesis.
出处 《中国全科医学》 CAS CSCD 北大核心 2015年第35期4378-4381,共4页 Chinese General Practice
关键词 雄激素迟钝综合征 诊断 治疗 Androgen-insensitivity syndrome Diagnosis Therapy
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