title: Response to LH-RH and HCG in two brothers with the Reifenstein syndrome.
abstract: 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.
mesh_terms: D015068:17-Ketosteroids / Q000652:urine; D000293:Adolescent; D006063:Chorionic Gonadotropin; D012734:Disorders of Sex Development / Q000235:genetics*; D005640:Follicle Stimulating Hormone / Q000097:blood* / Q000652:urine; D007987:Gonadotropin-Releasing Hormone; D006177:Gynecomastia; D006801:Humans; D007021:Hypospadias; D007248:Infertility, Male; D007986:Luteinizing Hormone / Q000097:blood*; D008297:Male; D011627:Puberty; D013739:Testosterone / Q000097:blood*
chemical_list: D015068:17-Ketosteroids; D006063:Chorionic Gonadotropin; D007987:Gonadotropin-Releasing Hormone; D013739:Testosterone; D007986:Luteinizing Hormone; D005640:Follicle Stimulating Hormone