Flatau E, Josefsberg Z, Prager-Lewin R, Markman-Halabe E, Kaufman H, Laron Z
Helv Paediatr Acta. 1976 Jan;30(4-5):377-83.
Two brothers with Reifenstein syndrome underwent LH-RH and HCG tests at various ages ranging from 13 to 17 years. We found that at age 13 the plasma LH and FSH response to one LH-RH injection was normal. After the age of 14, the basal plasma concentration of LH and FSH and their response to LH-RH became elevated. Concomitantly the plasma testosterone levels rose to abnormal levels. These findings are compatible with progressive development of primary gonadal dysfunction and with peripheral insensitivity to testosterone.
两名患有赖芬斯坦综合征的兄弟在13至17岁的不同年龄段接受了促黄体生成素释放激素(LH-RH)和人绒毛膜促性腺激素(HCG)测试。我们发现,13岁时,一次LH-RH注射后血浆促黄体生成素(LH)和促卵泡生成素(FSH)的反应正常。14岁以后,LH和FSH的基础血浆浓度及其对LH-RH的反应升高。与此同时,血浆睾酮水平升至异常水平。这些发现与原发性性腺功能障碍的渐进性发展以及对睾酮的外周不敏感性相符。