title: Hypothalamic hypogonadism in congenital adrenal hypoplasia.
abstract: Congenital adrenal hypoplasia (CAHP) in its X-linked form is associated with hypogonadotropic hypogonadism (HH). A 23 year old man with this disorder received substitution therapy with gluco- and mineralocorticoids starting one week after birth and, recently, pulsatile subcutaneous GnRH treatment via a miniature infusion pump with stepwise increasing doses from 50 to 200 ng/kg body weight/2 hours for a total of 394 days. Testosterone levels increased from prepubertal levels to 409 ng/dl after 2 weeks and to 626 ng/dl after 3 months of treatment. The results of pulsatile GnRH therapy in our patient prove the hypogonadotropic hypogonadism to be of hypothalamic origin. Pulsatile GnRH substitution is a successful therapeutic regimen in patients with CAHP leading to pituitary and gonadal maturation.
authors.affiliation: Universitäts-Kinderklinik, Abteilung für Endokrinologie, Universität Kiel, Germany.
authors.forename: C J
authors.initials: CJ
authors.lastname: Partsch
authors.forename: W G
authors.initials: WG
authors.lastname: Sippell
mesh_terms: D000311:Adrenal Glands / Q000002:abnormalities*; D000328:Adult; D000735:Androstenedione / Q000097:blood; D005640:Follicle Stimulating Hormone / Q000097:blood; D006801:Humans; D007006:Hypogonadism / Q000150:complications* / Q000188:drug therapy / Q000209:etiology; D007027:Hypothalamic Diseases / Q000150:complications* / Q000188:drug therapy; D007260:Infusion Pumps; D007986:Luteinizing Hormone / Q000097:blood; D008297:Male; D010906:Pituitary Hormone-Releasing Hormones / Q000008:administration & dosage / Q000627:therapeutic use; D013739:Testosterone / Q000097:blood
chemical_list: D010906:Pituitary Hormone-Releasing Hormones; D013739:Testosterone; D000735:Androstenedione; D007986:Luteinizing Hormone; D005640:Follicle Stimulating Hormone