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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。
Objective To summarize the basic characteristics and different variations of patients with complete androgen insensitivity syndrome (CAIS) in China. Methods The clinical manifestations, hormones and gonadopathological findings of 28 CAIS patients admitted to Peking Union Medical College Hospital from March 1976 to February 2002 were retrospectively studied. The results were compared with those in Chinese normal population and those reported in foreign countries For comparative analysis. Results The CAIS patients were female phenotype with an average height of 16 6 67 ± 3 81 cm, pubic hair and armpit hair scarce or absent, with breast development, female genitalia and vaginal blind end with a depth of (5 76 ± 1 64 ) cm, no cervix; testosterone levels decreased in 21.6% of the patients with combined testicular tumors; testicular tumors were found in 19%, large testicular hydrocele in 115%, dysplastic uterus in 192%. Conclusions The clinical manifestations of CAIS are more specific, but there are still some heterogeneity, including the presence of dysplastic uterus. The combined testosterone level is decreased. The incidence of CAIS in Chinese patients is higher than that reported abroad.