睾丸女性化综合征,在1817年首先由Steglehner报告,1953年Morris进一步研究建议使用该术语。该病发生率占出生婴儿的1/1万～1/5万,或相当于全部性分化障碍的5%。患儿核型为46XY。对雄性激素完全失去敏感性,发育成女性表现型。这种女性阴道短没有阴道口,有睾丸,位于在腹腔内、腹股沟内或大阴唇,生殖器表现性别难辩。到青春期有女性第二性 Testicular feminization syndrome, first reported by Steglehner in 1817, was further suggested by Morris in 1953 to use the term. The incidence of the disease accounted for 1/100 000 to 1/50 000 birth babies, or equivalent to 5% of all sex differentiation disorders. Children with karyotype 46XY. Male hormone completely lost the sensitivity to develop into a female phenotype. This female vagina short no vaginal mouth, testicular, located in the abdominal cavity, groin or labia majora, genital sex difficult to discern. Female adolescent secondary sex