[Abnormal hypothalamic-hypophyseal regulation as a new endocrine sign in the testicular feminisation syndrome (author's transl)].
In a 38-year-old woman with the testicular feminisation syndrome (TFS) there were increased serum levels of LH and FSH, despite normal testosterone levels, ranging from 808 to 1330 ng/dl. The patient exhibited complete end-organ resistance to androgens, as well as decreased free testosterone fraction of 1.5 percent. On stimulation with LH-RH there was enhanced LH reaction to 15 ng/ml, while FSH levels remained unchanged. Fluoxymesterone did not suppress LH and testosterone, as it did in normal controls. But the functional Leydig-cell reserve could be normally stimulated with HCG. Our results apparently confirm the suspected reduction in sensitivity of the hypothalamic-pituitary axis to androgens and suggest that this plays an important part in the pathogenesis of TES.
D000328:Adult; D013734:Androgen-Insensitivity Syndrome / Q000097:blood / Q000503:physiopathology*; D000728:Androgens / Q000378:metabolism; D006063:Chorionic Gonadotropin / Q000494:pharmacology; D003864:Depression, Chemical; D004054:Diethylstilbestrol / Q000494:pharmacology; D013196:Dihydrotestosterone / Q000097:blood; D005260:Female; D005474:Fluoxymesterone / Q000494:pharmacology; D005640:Follicle Stimulating Hormone / Q000097:blood; D007987:Gonadotropin-Releasing Hormone / Q000008:administration & dosage / Q000494:pharmacology; D006801:Humans; D007030:Hypothalamo-Hypophyseal System / Q000503:physiopathology*; D007275:Injections, Intravenous; D007985:Leydig Cells / Q000187:drug effects; D007986:Luteinizing Hormone / Q000097:blood; D008297:Male; D011863:Radioimmunoassay; D013268:Stimulation, Chemical; D013577:Syndrome; D013739:Testosterone / Q000097:blood
D000728:Androgens; D006063:Chorionic Gonadotropin; D013196:Dihydrotestosterone; D007987:Gonadotropin-Releasing Hormone; D013739:Testosterone; D004054:Diethylstilbestrol; D007986:Luteinizing Hormone; D005640:Follicle Stimulating Hormone; D005474:Fluoxymesterone
Hypothalamisch-hypophysäre Regulationsstörung als neues endokrinologisches Merkmal bei dem Syndrom der testikulären Feminisierung