【摘要】 目的　总结中国完全型雄激素不敏感综合征 (CAIS)患者的基本特征和不同变异。方法　回顾性研究了 1976年 3月至 2 0 0 2年 2月北京协和医院收治的 2 8例CAIS患者的临床表现、激素测定和性腺病理结果 ,并与中国正常人群和国外文献报道的CAIS患者进行比较分析。结果　CAIS患者为女性表型 ,成年身高 (16 6 6 7±3 81)cm ,阴毛、腋毛稀少或缺如 ,有乳房发育 ,女性外阴 ,阴道呈盲端 ,深度为 (5 76± 1 6 4 )cm ,无宫颈 ;2 6 1%合并存在睾酮水平下降 ;19 2 %出现睾丸肿瘤 ,11 5 %有较大的睾丸鞘膜积液 ,19 2 %存在有发育不良的子宫。结论　CAIS临床表现较为特异 ,但仍存在异质性 ,包括存在发育不良的子宫、合并存在睾酮水平下降 ;中国CAIS患者肿瘤的发生率高于国外的报道。更多还原

【Abstract】 Objective To summarize the clinical characteristics and varieties of complete androgen ins ensitivity syndrome(CAIS)in Chinese. Methods 28 cases of CAIS admitted to Peking Union Medical College Hospital from March of 1976 to February of 2002 were studied retrospectively through analyzing and com paring clinical characteristics,hormone levels and gonad pathological results wi th normal Chinese and CAIS patients reported in foreign literatures.Results CAIS patients were female habitus with (166.67±3.81)cm of adult height.There was absent or scanty axillary and pubic hair with gynecomastia.The external geni tala was female.There was a blind-ending vagina of (5.76±1.64)cm in length and no cervix.26.1% cases had lower testosterone level than that of normal male .19.2% had tumors in testis;11.5% had relatively large hydrocele testis;19.2% had rud imentary uteri. Conclusion The clinical manifestation of CAIS is rather specific but might be variable.It m ight have lower testosterone level than normal men and rudimentary uteri.Chinese CAIS has higher incidence of tumors in testis than reported elsewhere.更多还原