Reversal of idiopathic hypogonadotropic hypogonadism.

Détails

ID Serval
serval:BIB_7785BBF5EDE0
Type
Article: article d'un périodique ou d'un magazine.
Collection
Publications
Titre
Reversal of idiopathic hypogonadotropic hypogonadism.
Périodique
New England Journal of Medicine
Auteur(s)
Raivio T., Falardeau J., Dwyer A., Quinton R., Hayes F.J., Hughes V.A., Cole L.W., Pearce S.H., Lee H., Boepple P., Crowley W.F., Pitteloud N.
ISSN
1533-4406 (Electronic)
ISSN-L
0028-4793
Statut éditorial
Publié
Date de publication
2007
Peer-reviewed
Oui
Volume
357
Numéro
9
Pages
863-873
Langue
anglais
Notes
Publication types: Journal Article, pdf: Original Article
Résumé
BACKGROUND: Idiopathic hypogonadotropic hypogonadism, which may be associated with anosmia (the Kallmann syndrome) or with a normal sense of smell, is a treatable form of male infertility caused by a congenital defect in the secretion or action of gonadotropin-releasing hormone (GnRH). Patients have absent or incomplete sexual maturation by the age of 18. Idiopathic hypogonadotropic hypogonadism was previously thought to require lifelong therapy. We describe 15 men in whom reversal of idiopathic hypogonadotropic hypogonadism was sustained after discontinuation of hormonal therapy.
METHODS: We defined the sustained reversal of idiopathic hypogonadotropic hypogonadism as the presence of normal adult testosterone levels after hormonal therapy was discontinued.
RESULTS: Ten sustained reversals were identified retrospectively. Five sustained reversals were identified prospectively among 50 men with idiopathic hypogonadotropic hypogonadism after a mean (+/-SD) duration of treatment interruption of 6+/-3 weeks. Of the 15 men who had a sustained reversal, 4 had anosmia. At initial evaluation, 6 men had absent puberty, 9 had partial puberty, and all had abnormal secretion of GnRH-induced luteinizing hormone. All 15 men had received previous hormonal therapy to induce virilization, fertility, or both. Among those whose hypogonadism was reversed, the mean serum level of endogenous testosterone increased from 55+/-29 ng per deciliter (1.9+/-1.0 nmol per liter) to 386+/-91 ng per deciliter (13.4+/-3.2 nmol per liter, P<0.001), the luteinizing hormone level increased from 2.7+/-2.0 to 8.5+/-4.6 IU per liter (P<0.001), the level of follicle-stimulating hormone increased from 2.5+/-1.7 to 9.5+/-12.2 IU per liter (P<0.01), and testicular volume increased from 8+/-5 to 16+/-7 ml (P<0.001). Pulsatile luteinizing hormone secretion and spermatogenesis were documented.
CONCLUSIONS: Sustained reversal of normosmic idiopathic hypogonadotropic hypogonadism and the Kallmann syndrome was noted after discontinuation of treatment in about 10% of patients with either absent or partial puberty. Therefore, brief discontinuation of hormonal therapy to assess reversibility of hypogonadotropic hypogonadism is reasonable. (ClinicalTrials.gov number, NCT00392756 [ClinicalTrials.gov].).
Mots-clé
Adolescent, Adult, Extracellular Matrix Proteins/genetics, Follow-Up Studies, Gonadotropin-Releasing Hormone/deficiency, Gonadotropin-Releasing Hormone/therapeutic use, Gonadotropins/blood, Gonadotropins/therapeutic use, Humans, Hypogonadism/blood, Hypogonadism/congenital, Kallmann Syndrome/blood, Kallmann Syndrome/drug therapy, Male, Mutation, Nerve Tissue Proteins/genetics, Pedigree, Prospective Studies, Puberty, Delayed, Receptor, Fibroblast Growth Factor, Type 1/genetics, Receptors, G-Protein-Coupled/genetics, Receptors, LHRH/genetics, Remission, Spontaneous, Testosterone/blood, Testosterone/deficiency
Pubmed
Création de la notice
03/12/2014 16:34
Dernière modification de la notice
20/08/2019 15:34
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