Salvio Gianmaria, Balercia Giancarlo, Kadioglu Ates
Endocrinology Clinic, Department of Clinical and Molecular Sciences, Polytechnic University of Marche, Ancona 60126, Italy.
Section of Andrology, Department of Urology, Istanbul School of Medicine, Istanbul 34093, Türkiye.
Asian J Androl. 2025 May 1;27(3):322-329. doi: 10.4103/aja202483. Epub 2024 Oct 22.
Hypogonadotropic hypogonadism (HH) represents a relatively rare cause of nonobstructive azoospermia (NOA), but its knowledge is crucial for the clinical andrologists, as it represents a condition that can be corrected with medical therapy in 3 quarters of cases. There are forms of congenital HH, whether or not associated with an absent sense of smell (anosmic HH or Kallmann syndrome, and normosmic HH, respectively), and forms of acquired HH. In congenital HH, complete absence of pubertal development is characteristic. On the other hand, if the deficit occurs after the time of pubertal development, as in acquired HH patients, infertility and typical symptoms of late-onset hypogonadism are the main reasons for seeking medical assistance. Gonadotropin-releasing hormone (GnRH) or gonadotropin replacement therapy is the mainstay of drug therapy and offers excellent results, although a small but significant proportion of patients do not achieve sufficient responses.
低促性腺激素性性腺功能减退(HH)是导致非梗阻性无精子症(NOA)的一个相对罕见的原因,但了解它对于临床男科学医生至关重要,因为在四分之三的病例中,这是一种可以通过药物治疗纠正的病症。HH有先天性形式,无论是否伴有嗅觉缺失(分别为嗅觉缺失性HH或卡尔曼综合征,以及嗅觉正常性HH),还有后天性HH形式。在先天性HH中,青春期发育完全缺失是其特征。另一方面,如果缺陷发生在青春期发育之后,如后天性HH患者,不孕和迟发性性腺功能减退的典型症状是寻求医疗帮助的主要原因。促性腺激素释放激素(GnRH)或促性腺激素替代疗法是药物治疗的主要手段,且疗效显著,不过有一小部分但比例可观的患者并未获得足够的反应。