完全性雄激素不敏感性综合征(com-plete androgen insensitivity syndrome,CAIS)或称睾丸女性化综合征是最常见的男性假两性畸形,由于雄激素在靶细胞中受体性质上的异常和/或影响致病基因表现度的较件不同,不能发挥应有的男性化等作用。患者的表型在胚胎期就沿着女性化固有的倾向发展。临床上区别为以女性表型为主或以男性表型为主的两大类型,前者即完 Com- plete androgen insensitivity syndrome (CAIS), or testicular feminization syndrome, is the most common male pseudohermaphroditism because of androgen receptor abnormalities in target cells and / Or affect the performance of the disease-causing genes than the different parts, can not play its role as masculine. The patient’s phenotype develops during the embryo’s feminine tendencies. Clinically, the difference is mainly of female phenotype or male phenotype of the two major types, the former is the end