46,XY性腺发育不全或“纯”性腺发育不全是一种综合征(由于缺乏Turner体征),病人具有女性体型或隐睾体征、体毛稀疏、性激素升高。许多病人外阴正常,但一些病人阴蒂明显增大,另一些病人阴蒂发育不全。阴道狭窄或小,子宫和输卵管发育不良或正常。此综合征的明显特征是性腺肿瘤的发生率高,常倾向于青春期后发病,一些病人有乳腺发育。事实上,46,XY性腺发育不全病人的乳腺发育的发生率高,这就提出一个问题,在此种病例中女性化是否可表明索条状 46, XY gonadal dysgenesis or “pure” gonadal dysgenesis is a syndrome (due to the lack of Turner signs), the patient has female body or cryptorchidism signs, sparse body hair, elevated sex hormones. Many patients have normal vulva, but some patients have a significantly increased clitoris, while others have clitoral hypoplasia. Vaginal stenosis or small, uterus and fallopian tube dysplasia or normal. The obvious feature of this syndrome is the high incidence of gonadal tumors, often prone to onset after puberty, some patients have breast development. In fact, 46, XY glandular dysplasia in patients with high incidence of breast development, which raises the question of whether feminization in such cases can indicate the cable