一例真两性畸形的临床、细胞遗传学、组织学、免疫学及激素研究（作者译）

[Clinical, cytogenetical, histological, immunological and hormonal studies in a case of true hermaphroditism (author's transl)].

作者信息

Roger M, Roy C, Fellous M, Boccon-Gibod L, Pasqualini J R, Bonno R, Harpey J P

出版信息

Ann Endocrinol (Paris). 1980 Jan-Feb;41(1):73-5.

Abstract

A true hermaphrodite with ambiguous genitalia and 46 XX caryotype was investigated during adolescence. At 13 years testosterone concentrations (ng/ml) were 6 and 390 respectively in peripheral and right ovotestis venous blood. After removal of the right gonad, large fluctuations of estradiol levels (40 to 220 pg/ml) were observed. But the testosterone secretion by the left ovotestis was low, although responsive to hCG. A significant LH surge was induced by ethinyl-estradiol load before removal of the left gonad. The 5 alpha-reductase activity was normal in the pubic skin and the levels of cytosolic receptors of testosterone and DHT were high in the left gonad. The presence of H-Y antigen was demonstrated on lymphocytes.

摘要

一名患有两性生殖器畸形且核型为46 XX的真性雌雄同体患者在青春期接受了检查。13岁时，外周血和右侧卵睾静脉血中的睾酮浓度（ng/ml）分别为6和390。切除右侧性腺后，观察到雌二醇水平大幅波动（40至220 pg/ml）。尽管左侧卵睾对人绒毛膜促性腺激素有反应，但其睾酮分泌量较低。在切除左侧性腺之前，炔雌醇负荷诱导了显著的促黄体生成素高峰。耻骨皮肤中的5α-还原酶活性正常，左侧性腺中睾酮和双氢睾酮的胞质受体水平较高。淋巴细胞上检测到H-Y抗原的存在。