Fernández-Lazala R, Cabezas A, Fonseca M E, Murrieta S, Canales E S, Zárate A
J Endocrinol Invest. 1979 Jul-Sep;2(3):291-5. doi: 10.1007/BF03350419.
Dynamic testing of the hypothalamic pituitary system in familial hypogonadotropic hypogonadism has suggested the possibility of a central nervous disorder associated with variable pituitary secretory secretion. In order to investigate this issue further 7 patients of two families were studied. Male patients exhibited two types of FSH responses to LHRH administration. Two patients had a small response, and the other exhibited a normal response. The LH response was absent or small in both male and female patients. One month therapy with daily injections of LHRH did not improve pituitary responsiveness to a second LHRH test. Estrogen treatment given to females during one month had no improvement effect on another LHRH test. Estradiol benzoate produced a surge in both LH and FSH in half of patients tested which is surprising in the presence of a previous clomiphene negative response. Two of four women had elevated prolactin levels which increased defectuously after metoclopramide administration which could suggest an abnormal hypothalamic status. On the basis of these results it seems that familial hypogonadotropism is originated by an abnormality at two levels, the anterior pituitary and the hypothalamus as well.
对家族性性腺功能减退性性腺功能减退症患者下丘脑 - 垂体系统的动态测试表明，可能存在与垂体分泌变化相关的中枢神经系统疾病。为了进一步研究这个问题，对两个家族的7名患者进行了研究。男性患者对促黄体生成素释放激素（LHRH）给药表现出两种促卵泡生成素（FSH）反应类型。两名患者反应较小，另一名表现出正常反应。男性和女性患者的促黄体生成素（LH）反应均缺失或较小。每日注射LHRH进行为期一个月的治疗，并未改善垂体对第二次LHRH测试的反应性。女性在一个月内接受雌激素治疗，对另一次LHRH测试没有改善作用。苯甲酸雌二醇在一半接受测试的患者中使LH和FSH均出现激增，这在之前氯米芬试验呈阴性反应的情况下令人惊讶。四名女性中有两名催乳素水平升高，在给予胃复安后异常升高，这可能提示下丘脑状态异常。基于这些结果，家族性性腺功能减退似乎源于两个层面的异常，即垂体前叶和下丘脑。
