Clinical Management of Congenital Hypogonadotropic Hypogonadism

Abstract

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Hypogonadism and Cryptorchidism

“…Congenital hypogonadotropic hypogonadism (CHH) is a condition characterized by a decreased production, secretion, or action of GnRH (123). The patients with this condition lack FSH and LH and display the resultant low testosterone level since fetal life, and therefore they can present with micropenis or cryptorchidism at birth (108,236,237). Low testosterone levels during puberty and adulthood result in absent or incomplete puberty, eunuchoid body proportions, decreased virilization, reduced libido, and sexual function, infertility, or osteoporosis from long-standing hypogonadism (123).…”

“…Early diagnosis allows pediatricians to plan for induction of puberty at the appropriate time preventing the potential pubertal delay (108). Since the mini-pubertal hormonal surge is associated with Sertoli cell proliferation (238), the lack of mini-puberty might relate to reduced Sertoli cell proliferation and the resultant decreased fertility later in life (237). Therefore, some studies investigated the role of replacement of deficient mini-pubertal hormones by using GnRH, or gonadotropins during infancy in patients with CHH to improve reproductive health, especially fertility, later in life (237).…”

“…Since the mini-pubertal hormonal surge is associated with Sertoli cell proliferation (238), the lack of mini-puberty might relate to reduced Sertoli cell proliferation and the resultant decreased fertility later in life (237). Therefore, some studies investigated the role of replacement of deficient mini-pubertal hormones by using GnRH, or gonadotropins during infancy in patients with CHH to improve reproductive health, especially fertility, later in life (237). However, only some of these studies included cryptorchid boys, and testicular descent was not the primary outcome of most of these studies, making it difficult to assess the effect of this approach on testicular descent (41, 133,[239][240][241][242][243].…”

Follicle-Stimulating Hormone (FSH) Action on Spermatogenesis: A Focus on Physiological and Therapeutic Roles

“…The reduced gonadotropin stimulation results in low sex hormone secretion and sperm production. There is compelling evidence that HH patients benefit from either GnRH or gonadotropin replacement therapy, which results in an improvement of both spermatogenesis and androgenisation [115,116].…”

FSH for the Treatment of Male Infertility

“…In the context of female fertility, several studies attempted to determine congenital and environmental issues modulating the response to FSH [45,46], aiming to improve assisted reproductive technologies (ART) outcomes [47]. In the male, deficiency of FSH signaling may impair fertility and requires clinical treatments for restoring the gonadal function [48]. For these purposes, a number of FSH molecules were developed to modulate the target cell response to the hormone and used for infertility treatments.…”