Response to LH-RH and clomiphene citrate in two women with the Prader-Labhart-Willi syndrome.
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.
D000328:Adult; D002239:Carbohydrate Metabolism, Inborn Errors / Q000097:blood / Q000188:drug therapy*; D002996:Clomiphene / Q000627:therapeutic use*; D004958:Estradiol / Q000097:blood; D005260:Female; D005640:Follicle Stimulating Hormone / Q000097:blood; D005500:Follow-Up Studies; D007987:Gonadotropin-Releasing Hormone / Q000627:therapeutic use*; D006801:Humans; D008607:Intellectual Disability / Q000097:blood / Q000188:drug therapy; D007986:Luteinizing Hormone / Q000097:blood*; D009765:Obesity / Q000097:blood / Q000188:drug therapy*; D013577:Syndrome; D013997:Time Factors
D002996:Clomiphene; D007987:Gonadotropin-Releasing Hormone; D004958:Estradiol; D007986:Luteinizing Hormone; D005640:Follicle Stimulating Hormone