Paulson D F, Wiebe H R, Hammond C B
Urology. 1975 Sep;6(3):333-6. doi: 10.1016/0090-4295(75)90758-x.
Hypogonadotropic hypogonadism has been identified as a cause of partial or complete failure of puberty, may be familial and may have other associated abnormalities of hyposmia, intellectual retardation, perceptive deafness, color blindness, skeletal deformities, and gynecomastia. Pituitary function is usually normal with the primary defect believed to be hypothalamic. A twenty-year-old white male with a clinical diagnosis of hypogonadotropic hypogonadism and anosmia under-went complete endocrine evaluation with evaluation of the pituitary response to luteinizing hormone-releasing hormone. FSH (follicle-stimulating hormone) and LH (luteinizing hormone) release after luteinizing hormone-releasing hormone did occur, but the response was less than that seen in normal controls. Evaluation demonstrated that the pituitary-gonadal axis was intact with the hypothalamic-pituitary axis being defective. Therapy with the synthetic decapeptide (luteinizing hormone-releasing hormone) is correct theoretically and may be superior to therapy with exogenous gonadotropins.
低促性腺激素性性腺功能减退已被确认为青春期部分或完全发育失败的一个原因，可能是家族性的，并且可能伴有其他相关异常，如嗅觉减退、智力迟钝、感知性耳聋、色盲、骨骼畸形和男性乳房发育。垂体功能通常正常，主要缺陷被认为在下丘脑。一名20岁的白人男性，临床诊断为低促性腺激素性性腺功能减退和嗅觉缺失，接受了全面的内分泌评估，包括评估垂体对促黄体生成素释放激素的反应。促黄体生成素释放激素刺激后促卵泡生成素（FSH）和促黄体生成素（LH）确实释放，但反应低于正常对照组。评估表明垂体-性腺轴完整，而下丘脑-垂体轴存在缺陷。理论上，使用合成十肽（促黄体生成素释放激素）进行治疗是正确的，并且可能优于使用外源性促性腺激素进行治疗。
