Congenital hypogonadotropic hypogonadism: implications of absent mini-puberty.

Détails

ID Serval
serval:BIB_11729E2919A9
Type
Article: article d'un périodique ou d'un magazine.
Sous-type
Synthèse (review): revue aussi complète que possible des connaissances sur un sujet, rédigée à partir de l'analyse exhaustive des travaux publiés.
Collection
Publications
Titre
Congenital hypogonadotropic hypogonadism: implications of absent mini-puberty.
Périodique
Minerva endocrinologica
Auteur(s)
Dwyer A.A., Jayasena C.N., Quinton R.
ISSN
1827-1634 (Electronic)
ISSN-L
0391-1977
Statut éditorial
Publié
Date de publication
06/2016
Peer-reviewed
Oui
Volume
41
Numéro
2
Pages
188-195
Langue
anglais
Notes
Publication types: Journal Article ; Review
Publication Status: ppublish
Résumé
The phenomenon known as "mini-puberty" refers to activation of the neonatal hypothalamo-pituitary axis causing serum concentrations of gonadotrophins and testosterone (T) to approach adult male levels. This early neonatal period is a key proliferative window for testicular germ cells and immature Sertoli cells. Although failure to spontaneously initiate (adolescent) puberty is the most evident consequence of a defective gonadotropin-releasing hormone (GnRH) neurosecretory network, absent mini-puberty is also likely to have a major impact on the reproductive phenotype of men with congenital hypogonadotrophic hypogonadism (CHH). Furthermore, the phase of male mini-puberty represents a key window-of-opportunity to identify congenital GnRH deficiency (either isolated CHH, or as part of combined pituitary hormone deficiency) in childhood. Among male neonates exhibiting "red flag" indicators for CHH (i.e. maldescended testes with or without cryptorchidism) a single serum sample (between 4-8 weeks of life) can pinpoint congenital GnRH deficiency far more rapidly and with much greater accuracy than dynamic tests performed in later childhood or adolescence. Potential consequences for missing absent mini-puberty in a male neonate include the lack of monitoring of pubertal progression/lack of progression, and the missed opportunity for early therapeutic intervention. This article will review our current understanding of the mechanisms and clinical consequences of mini-puberty. Furthermore, evidence for the optimal clinical management of patients with absent mini-puberty will be discussed.

Mots-clé
Gonadotropins/blood, Humans, Hypogonadism/complications, Infant, Newborn, Male, Puberty, Puberty, Delayed/etiology, Testosterone/blood
Pubmed
Web of science
Création de la notice
04/11/2016 13:42
Dernière modification de la notice
20/08/2019 12:39
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