Familial idiopathic gonadotropin deficiency: a hypothalamic form of hypogonadism.
To date, familial idiopathic gonadotropin deficiency (FIGD) has not been delineated as either a hypothalamic or a pituitary form of hypogonadism. Leydig cell sensitivity to human chorionic gonadotropin (HCG) has also been suggested as subnormal in FIGD. Also, in a few previously reported families the Kallmann syndrome was not clearly ruled out. Data herewith reported on three sibs with FIGD supported the following conclusions: 1) FIGD is due to insufficient hypothalamic luteinizing hormone-releasing hormone (LRH) secretion, 2) the sensitivity of Leydig cells to HCG is normal, 3) LRH treatment may be helpful in these patients, 4) an associated hypothalamic-pituitary-prolactin (PRL) dysfunction may also be present, and 5) FIGD and the Kallmann syndrome are different entities having a similar pathophysiology but different cause and overall clinical picture.
D005260:Female; D005640:Follicle Stimulating Hormone / Q000172:deficiency; D007987:Gonadotropin-Releasing Hormone / Q000172:deficiency; D006062:Gonadotropins / Q000172:deficiency*; D006801:Humans; D007006:Hypogonadism / Q000235:genetics*; D007031:Hypothalamus / Q000002:abnormalities*; D007986:Luteinizing Hormone / Q000172:deficiency; D008297:Male; D012727:Sex Characteristics; D013577:Syndrome
D006062:Gonadotropins; D007987:Gonadotropin-Releasing Hormone; D007986:Luteinizing Hormone; D005640:Follicle Stimulating Hormone