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低促性腺激素性性功能减退(HH)是一种可发生于两性的临床综合征。其外周血中促性腺激素(Gn)正常或略低,而性腺却无功能,长期以来使临床医师迷惑不解。本文用多次取样测定Gn水平的方法做为衡量下丘脑促性腺激素释放素(GnRH)分泌的指标,验证了以下假说:本病代表了多种形式的内源性GnRH脉冲式释放异常。在对正常男女进行广泛研究并建立正常值后,发现HH男女患者中,有GnRH分泌量的异常,包括GnRH分泌完全缺如、分泌的幅度和频率有缺陷,以及释放的Gn生物活性异常。此外,采用符合生理的外源性GnRH替代治疗方案以模拟内源性GnRH分泌的正常频率,可使低Gn性男女患者生殖和生育功能完全恢复正常。因此,本病的异型性及其对GnRH满意的临床和生化反应提示其基本缺陷是下丘脑部分或完全性合成和/或释放GnRH障碍。维持内源性GnRH分泌的生理频率和幅度对正常生殖功能是必不可少的。
Hypogonadotropic hypofunction (HH) is a clinical syndrome that can occur in both sexes. Its peripheral blood gonadotropin (Gn) is normal or slightly lower, while the gonads have no function, for a long time puzzled clinicians. In this paper, the method of determining the level of Gn by multiple sampling was used as a measure of the secretion of gonadotropin-releasing hormone (GnRH) in hypothalamus, and the following hypothesis was verified: This disease represents various forms of endogenous GnRH pulsed release anomalies. After extensive research and normalization of normal men and women, abnormal GnRH secretion was found in both men and women with HH, including complete absence of GnRH secretion, defects in the magnitude and frequency of secretion, and abnormalities of released Gn biological activity. In addition, reproductive and reproductive functions of men and women with low Gn can be fully restored using a physiologically-compatible alternative GnRH regimen to mimic the normal frequency of endogenous GnRH secretion. Therefore, the atypia of this disease and its clinical and biochemical response to GnRH suggest that its underlying defect is the partial or complete synthesis and / or release of GnRH disorders in the hypothalamus. Maintaining the physiological frequency and magnitude of endogenous GnRH secretion is essential for normal reproductive function.