James C S, Pride S M, Yuen B H
Department of Obstetrics and Gynecology, Faculty of Medicine, University of British Columbia, Grace Hospital, Vancouver, Canada.
Obstet Gynecol. 1990 Nov;76(5 Pt 2):921-4. doi: 10.1097/00006250-199011000-00046.
A 30-year-old woman with primary amenorrhea, hypothalamic hypogonadism, decreased sense of smell, and primary infertility failed to respond to pulsatile exogenous gonadotropin-releasing hormone. In addition, failure to respond to stimulation with human menopausal gonadotropins was consistent with concomitant ovarian failure. Perturbation of normal cellular migration during embryogenesis in the regions of the olfactory placode, yolk sac, hindgut, and gonadal ridge may explain both the hypothalamic defect and ovarian failure experienced by this woman. She demonstrates that gonadal failure need not be accompanied by elevated gonadotropin levels; nor do low gonadotropin levels necessarily indicate potentially responsive ovaries. These findings are consistent with the coexistence of isolated gonadotropin deficiency and ovarian failure in the same individual.
一名30岁女性,患有原发性闭经、下丘脑性性腺功能减退、嗅觉减退和原发性不孕,对外源性促性腺激素释放激素脉冲治疗无反应。此外,对人绝经期促性腺激素刺激无反应,提示合并卵巢功能衰竭。胚胎发育过程中,嗅基板、卵黄囊、后肠和性腺嵴区域正常细胞迁移受到干扰,可能解释了该女性的下丘脑缺陷和卵巢功能衰竭。她表明,性腺功能衰竭不一定伴有促性腺激素水平升高;促性腺激素水平低也不一定意味着卵巢有反应。这些发现与同一个体中孤立性促性腺激素缺乏和卵巢功能衰竭并存相一致。