[Turner's phenotype in a male with deficit of ACTH and gonadotropins (author's transl)].
A case of male Turner's syndrome in a 23-year-old patient is reported. Clinical features included total eunuchoidism, shield-like chest, cubitus valgus, lymphedema in the extremities (hands and feet) and a shortened fourth metacarpal. Hormonal studies revealed very low levels of gonadotropins, cortisol, testosterone and HGH, and normal values for PRL and TSH. Gonadotropin levels did not change after the administration of 100 micrograms LH-RH and 500 micrograms LH-RH every 8 hours during 5 days. Testosterone levels increased when HCG was given. Deficit of ACTH release was demonstrated after the administration of metopyrone, ACTH and 0.1 UI insulin per kilogram of body weight to induce hypoglycemia. Hypoglycemia with insulin and arginine did not determine an increase of GH levels, instead of previous estrogen therapy. These results reveal a hypophyseal hormonal defect in relation to ACTH, LH, FSH and GH release. Hormone abnormalities found in the present case have not been previously described.
D000324:Adrenocorticotropic Hormone / Q000172:deficiency* / Q000627:therapeutic use; D000328:Adult; D006063:Chorionic Gonadotropin / Q000172:deficiency* / Q000627:therapeutic use; D006801:Humans; D008297:Male; D009634:Noonan Syndrome / Q000175:diagnosis* / Q000188:drug therapy
D006063:Chorionic Gonadotropin; D000324:Adrenocorticotropic Hormone
Fenotipo Turner en un varón con déficit de gonadotropinas y ACTH.