Treatment of congenital hypogonadotropic hypogonadism in male patients

Abstract

Congenital hypogonadotropic hypogonadism (CHH) is characterized by complete or partial failure of pubertal development because of inadequate secretion of gonadotropic hormones. CHH consists of hypogonadotropic hypogonadism with anosmia or hyposmia, Kallmann syndrome, and the normosmic variation normosmic idiopathic hypogonadotropic hypogonadism. CHH is one of the few treatable diseases of male infertility, although men with primary testicular dysfunction have irreversibly diminished spermatogenic capacity. The approach to CHH treatment is determined by goals such as developing virilization or inducing fertility. This review focuses on the current knowledge of therapeutic modalities for inducing puberty and fertility in men with CHH.

Are they functional hypogonadal men? Testosterone serum levels unravel male idiopathic infertility subgroups

“…While reduced testosterone serum levels reflect reduced LH stimulation on Leydig cells, altered spermatogenesis is the result of the impairment of intra-testicular testosterone levels and/or FSH stimulation on Sertoli cells. Since this condition is caused by suboptimal testicular stimulation by gonadotropins, patients with hypogonadotropic hypogonadism could be effectively treated with exogenous gonadotropins or gonadotropin-releasing hormone (GnRH) with a significant testosterone raise and semen analysis improvement [ 45 – 47 ]. Alongside hypogonadotropic hypogonadism, testicular stimulation with gonadotropins could have other fields of application.…”

Diagnosis and Management of Osteoporosis in Children and Adolescents

“…In cases of secondary osteoporosis, treatment of the underlying disease and cessation of osteotoxic medication are prioritized. Hormonal deficiencies should be diagnosed as early as possible and adequately managed [24,25]. Although conservative management is preferred in children and adolescents, pharmacological therapy should be considered in patients with recurrent lone bone fractures or vertebral fractures [7].…”

A novel variant in NR0B1 causing X-linked adrenal hypoplasia congenita

“…The disrupted hypothalamic-pituitary-gonadal axis in AHC leads to decreased follicle-stimulating hormone (FSH) and luteinizing hormone secretion, impairing gonadal function, delaying or preventing puberty, and causing infertility [ 14 ]. Treatment options include gonadotropin-releasing hormone pulsatile therapy, sequential gonadotropin therapy with FSH and human chorionic gonadotropin, and assisted reproductive technologies like testicular sperm extraction associated with intracytoplasmic sperm injection [ 15 ].…”