摘要
目的总结睾丸退化患者的临床特征和鉴别诊断方法。方法回顾性总结了5例睾丸退化患者的临床表现、激素测定和性腺病理结果。结果睾丸退化患者社会性别均为女性,多数因外生殖器性别模糊或青春期后原发闭经而就诊,患者染色体核型为46,XY、盆腔检查无子宫和宫颈,青春期后缺乏女性第二性征发育、促性腺激素水平升高、性腺水平低下、人绒毛膜促性腺激素(hCG)刺激试验睾酮无增加,性腺均为发育不良的睾丸或条索状组织。结论睾丸退化患者临床较为罕见,其病因尚不清楚,临床上需注意与其他性发育异常患者鉴别。
Objectives： To summarize the clinical characteristics and differential diagnosis of testicular regression.
Methods： Five cases of testicular regression, with their clinical manifestations, hormones levels and gonad pathology were analyzed retrospectively. Methods for differential diagnosis were discussed.
Results.The patients with testicular regression showed a female social sex. The chief complaints were ambiguous genitalia after birth or with primary amenorrhea after puberty. They were all 46,XY chromo- somes without uterus and female secondary sexual characteristics. The gonadotropin level was elevated and gonadal hormone level was reduced and there was no response of testosterone increase to hCG stimulation after puberty. The pathology of gonads showed dysgenetic testes or streak in nature.
Conclusions. Testicular regression is a very rare kind of disorders of sex development. Its etiology is unknown yet and it should be differentiated with other disorders of sex development.
出处
《生殖医学杂志》
CAS
2008年第3期178-182,共5页
Journal of Reproductive Medicine
