成人发生的特发性低促性腺激素性性腺功能减退症研究进展

Research Progress of Adult-Onset Idiopathic Hypogonadotropic Hypogonadism

摘要: 男性的促性腺激素(gonadotropins)包括卵泡刺激素(follicle stimulating hormone, FSH)及黄体生成激素(luteinizing hormone, LH)，下丘脑分泌的促性腺激素释放激素(gonadotropin releasing hormone, GnRH)可兴奋垂体分泌LH及FSH。男性特发性低促性腺激素性性腺功能减退症(idiopathic hypogonadotropic hypogonadism, IHH)是一种先天性疾病，表现为睾丸小，阴茎短，没有青春期发育。然而，一些经历过完整青春期发育的成年男性，也可出现特发性的低促性腺激素性性腺功能减退症，目前其发病原因未明，可能与下丘脑GnRH神经元的功能缺失有关，临床表现为性欲减退、勃起障碍和男性乳房发育，治疗上，可给予脉冲性GnRH治疗，促进促性腺激素分泌的恢复和精子的产生。本文旨在对成人发生的特发性低促性腺激素性性腺功能减退症的发病机制、临床特点、诊断与治疗进行综述，以便更好地为临床和科研提供依据。

Abstract: Male gonadotropins include follicle stimulating hormone (FSH) and luteinizing hormone (LH). Gonadotropin releasing hormone (GnRH) secreted by hypothalamus can excite pituitary gland to secrete LH and FSH. Male idiopathic hypogonadotropic hypogonadism (IHH) is a congenital disease characterized by small testicles, short penis and no puberty. However, isolated hypogonadotropic hypogonadism can also occur in some adult men who have experienced complete puberty. At present, the cause of its pathogenesis is unknown, which may be related to the loss of function of GnRH neurons in hypothalamus. The clinical manifestations are hypolibido, erectile dysfunction (ED) and male breast development. In treatment, pulsed GnRH can be given to promote the recovery of gonadotropin secretion and sperm production. This paper aims to review the pathogenesis, clinical characteristics, diagnosis and treatment of adult-onset idiopathic hypogonadotropic hypogonadism, so as to provide a better basis for clinical and scientific research.