Neonatal gonadotropin therapy in male congenital hypogonadotropic hypogonadism.
Congenital hypogonadotropic hypogonadism (CHH) causes pubertal failure and infertility in both women and men due to partial or total secretory failure of the two pituitary gonadotropins lutropin (LH) and follitropin (FSH) during periods of physiological activation of the gonadotropic axis. Men and women with CHH frequently seek treatment for infertility after hypogonadism therapy. Some etiologies, such as autosomal dominant or X-linked Kallmann syndrome, raise the question of hereditary transmission, leading to increasing demands for genetic counseling and monitoring of medically assisted pregnancies. Diagnosis and treatment of newborn boys is, therefore, becoming an increasingly important issue. In male individuals with complete forms of CHH, the antenatal and neonatal gonadotropin deficit leads to formation of a micropenis and cryptorchidism, which could undermine future sexual and reproductive functions. Standard treatments, usually started after the age of puberty, often only partially correct the genital abnormalities and spermatogenesis. The aim of this Review is to examine the possible additional benefits of neonatal gonadotropin therapy in male patients with CHH. Encouraging results of neonatal therapy, together with a few reports of prepubertal treatment, support the use of this novel therapeutic strategy aimed at improving sexual and reproductive functions in adulthood.
Departement de Pédiatrie Endocrinienne, Hôpital Bicêtre-University Paris-Sud, 78 Rue du Général Leclerc, F-94275 Le Kremlin-Bicêtre, France.
Claire
C
Bouvattier
Luigi
L
Maione
Jérôme
J
Bouligand
Catherine
C
Dodé
Anne
A
Guiochon-Mantel
Jacques
J
Young
D000367:Age Factors; D000818:Animals; D007987:Gonadotropin-Releasing Hormone / Q000097:blood; D006065:Gonadotropins, Pituitary / Q000097:blood* / Q000494:pharmacology / Q000627:therapeutic use*; D006801:Humans; D007006:Hypogonadism / Q000097:blood* / Q000188:drug therapy*; D007231:Infant, Newborn; D008297:Male; D011627:Puberty / Q000097:blood / Q000187:drug effects; D013739:Testosterone / Q000097:blood
D006065:Gonadotropins, Pituitary; D007987:Gonadotropin-Releasing Hormone; D013739:Testosterone