Congenital hypogonadotropic hypogonadism and Kallmann syndrome as models for studying hormonal regulation of human testicular endocrine functions.

摘要: Men with Kallmann syndrome (KS) and those congenital isolated hypogonadotropic hypogonadism normal olfaction share a chronic, usually profound deficit, in FSH LH, the two pituitary gonadotropins. Many studies indicate that this gonadotropin deficiency is already present during fetal life, thus explaining micropenis, cryptorchidism marked testicular hypotrophy at birth. In addition, neonatal activation of secretion compromised boys severe CHH/Kallmann, preventing first phase postnatal activation. Finally, CHH characterized by persistence, vast majority cases, time puberty adulthood. This prevents pubertal reactivation required for physiological sex steroid peptide production, spermatogenesis. CHH/KS represents pathological paradigm can help to unravel, vivo, role each human exocrine endocrine functions different stages development. Recombinant gonadotropins pure LH or activity have been used stimulate Leydig's cells Sertoli's cells, respectively, thereby clarify their paracrine interaction vivo. The effects these pharmacological probes be assessed measuring changes they provoke circulating hormone concentrations. review discusses impact chronic on interstitial compartment, which contains testosterone-, estradiol- INSL3-secreting cells. It also examines regulation inhibin B anti-Mullerian (AMH) seminiferous tubules, insights provided stimulation recombinant gonadotropins, either individually combination.