Familial idiopathic gonadotropin deficiency: a hypothalamic form of hypogonadism.

Abstract

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.

• Authors: S P Toledo, W Luthold, E Mattar
• Journal: American journal of medical genetics (Am J Med Genet) Vol. 15 Issue 3 Pg. 405-16 (Jul 1983) ISSN: 0148-7299 [Print] United States
• PMID: 6410916 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Gonadotropins
• Gonadotropin-Releasing Hormone
• Luteinizing Hormone
• Follicle Stimulating Hormone

Topics

• Female
• Follicle Stimulating Hormone (deficiency)
• Gonadotropin-Releasing Hormone (deficiency)
• Gonadotropins (deficiency)
• Humans
• Hypogonadism (genetics)
• Hypothalamus (abnormalities)
• Luteinizing Hormone (deficiency)
• Male
• Sex Characteristics
• Syndrome