title: Preclinical hypogonadism in genetic hemochromatosis in the early stage of the disease: evidence of hypothalamic dysfunction.
abstract: We studied endocrine functions at baseline and after TRH and LHRH stimulation in a group of 7 young male patients with genetic hemochromatosis (HE) without liver damage (i.e. fibrosis and cirrhosis). In five patients endocrine re-evaluations after complete iron depletion was also performed. Mean basal testosterone (T), FSH, LH and PRL were significantly lower than in controls. Serum T increased normally after HCG stimulation. The normal or high increments of LH after LHRH stimulation suggest that secretion capacity of LH was intact and that hypothalamic dysfunction could be responsible for the preclinical gonadal deficiency found in our patients. The response of PRL to TRH indicates that secretion capacity of lactotrophs although present, was decreased and did not improve after phlebotomy therapy. After iron depletion the two patients with the lowest basal T levels showed the highest increments indicating that in the early stages of hypothalamic-pituitary damage gonadal dysfunction is still reversible in HE patients.
authors.affiliation: Istituto di Scienze Biomediche S. Gerardo, Università di Milano, Monza, Italy.
authors.forename: A
authors.initials: A
authors.lastname: Piperno
authors.forename: M R
authors.initials: MR
authors.lastname: Rivolta
authors.forename: R
authors.initials: R
authors.lastname: D'Alba
authors.forename: S
authors.initials: S
authors.lastname: Fargion
authors.forename: F
authors.initials: F
authors.lastname: Rovelli
authors.forename: A
authors.initials: A
authors.lastname: Ghezzi
authors.forename: M
authors.initials: M
authors.lastname: Micheli
authors.forename: G
authors.initials: G
authors.lastname: Fiorelli
mesh_terms: D000328:Adult; D006063:Chorionic Gonadotropin; D005640:Follicle Stimulating Hormone / Q000097:blood; D007987:Gonadotropin-Releasing Hormone; D006432:Hemochromatosis / Q000150:complications* / Q000235:genetics / Q000503:physiopathology; D006801:Humans; D007006:Hypogonadism / Q000209:etiology*; D007031:Hypothalamus / Q000503:physiopathology*; D007986:Luteinizing Hormone / Q000097:blood; D008297:Male; D011388:Prolactin / Q000097:blood; D013739:Testosterone / Q000097:blood; D013973:Thyrotropin-Releasing Hormone
chemical_list: D006063:Chorionic Gonadotropin; D007987:Gonadotropin-Releasing Hormone; D013739:Testosterone; D013973:Thyrotropin-Releasing Hormone; D011388:Prolactin; D007986:Luteinizing Hormone; D005640:Follicle Stimulating Hormone