McGuffin W L, Rogol A D
J Clin Endocrinol Metab. 1975 Aug;41(2):325-31. doi: 10.1210/jcem-41-2-325.
Two women with the Prader-Labhart-Willi syndrome are presented. The gonadotropin response to LH-RH administration was studied prior to, immediately following, and 6 months after a 6-week trial of clomiphene citrate, 200 mg per day for 21 days in divided doses, followed by 100 mg for 21 days in divided doses, followed by 100 mg per day for 14 days in divided doses, and followed by 50 mg per day in a single dose for an additional 14 days. During therapy, the basal gonadotropin and estradiol concentrations rose from prepubertal levels to those of mature women in midmenstrual cycle. However, 6 months after cessation of treatment, the basal gondadotropin and estradiol levels had returned to the prepubertal range. The initial response to LH-RH in the 2 patients differed in that one was clearly prepubertal and the other indistinguishable from the broad range of the adult normal response. The LY and FSH responses to LH-RH administration was greater after 6 weeks of clomiphene citrate therapy than they were either before (both patients) or 6 months after treatment (1 patient). We conclude that there is heterogeneity in the response to LH-RH administration in the Prader-Labhart-Willi Syndrome, just as there is in other syndromes of hypogonadotropic hypogonadism. A normal adult response of gonadotropins to the administration of LH-RH was acheived during clomiphene citrate therapy.
本文报告了两名普拉德-拉巴尔特-威利综合征患者。在进行为期6周的枸橼酸氯米芬试验之前、试验刚结束时以及试验结束6个月后，研究了促性腺激素对促黄体生成素释放激素（LH-RH）给药的反应。枸橼酸氯米芬的给药方案为：每天200mg，分剂量服用21天，随后每天100mg，分剂量服用21天，再随后每天100mg，分剂量服用14天，最后每天50mg，单次服用14天。治疗期间，基础促性腺激素和雌二醇浓度从青春期前水平升至月经周期中期成熟女性的水平。然而，治疗停止6个月后，基础促性腺激素和雌二醇水平已恢复至青春期前范围。两名患者对LH-RH的初始反应不同，其中一名明显处于青春期前，另一名与广泛的成人正常反应难以区分。在接受枸橼酸氯米芬治疗6周后，LH和促卵泡生成素（FSH）对LH-RH给药的反应比治疗前（两名患者）或治疗后6个月（一名患者）更大。我们得出结论，普拉德-拉巴尔特-威利综合征患者对LH-RH给药的反应存在异质性，就像其他低促性腺激素性性腺功能减退综合征一样。在枸橼酸氯米芬治疗期间，促性腺激素对LH-RH给药达到了正常成人反应。
