论文部分内容阅读
本文报告28例患者。包括原发性性腺功能减退11例,其中性腺发育不全8例,Robindow综合征2例,真两性畸形1例。6例按男性抚养,5例接女性抚养。诊断依据在青春期后是LH和/或FSH增高,在青春期前是HCG刺激睾酮试验无反应或反应减低,或睾丸活检证明存在发育不全。不全性雄激素不敏感综合征(PAIS)1例,诊断是根据男性假两性畸形家族史,在儿童期及以后的自发青春期,经外源性雄激素刺激仍为男性化不良,成人期阴茎小于正常,促性腺激素水平增高,睾酮水平
This article reports 28 patients. Including the primary hypogonadism in 11 cases, including 8 cases of gonadal hypoplasia, Robindow’s syndrome in 2 cases, 1 case of hermaphroditism. 6 cases were raised by men and 5 cases were raised by women. The diagnosis is based on an increase in LH and / or FSH after puberty, with no response or response to HCG-stimulated testosterone testing before puberty, or testis biopsy evidence of hypoplasia. One case of incomplete androgen insensitivity syndrome (PAIS) was diagnosed based on the family history of male pseudohermaphroditism. In childhood and subsequent spontaneous adolescence, exogenous androgen stimulation was still masculine, and the adult penis was less than Normal, elevated gonadotropin levels, testosterone levels