女性肝豆状核变性下丘脑垂体卵巢轴功能研究

Research on the hypothalamic-pituitary-ovary axis function in female patients with hepatolenticular degeneration

目的 研究女性肝豆状核变性 (HLD)患者下丘脑 垂体 卵巢轴激素分泌功能。方法 放免法测定 13例HLD患者 (HLD组 )和 15名正常对照组血清垂体、性激素水平。结果 HLD患者卵泡刺激素 (FSH)显著低于正常组 (P <0 0 5 ) ,孕酮 (P)、黄体生成素 (LH)、雌二醇 (E2 )亦显著降低 (P <0 0 1) ,性激素结合球蛋白 (SHBG)则显著高于正常对照组 (P <0 0 0 1) ,10例患者行促性腺激素释放激素 (GnRH)试验 ,发现患者腺垂体对外源性GnRH的刺激反应迟钝。结论 HLD患者垂体、性腺细胞中部分酶的活性降低 ,使激素分泌功能降低 ;同时肝的铜沉积扰乱了蛋白代谢 ,使SHBG升高 ,进一步降低了性激素的生物效应。

Objective To study the hypothalamic-pituitary-ovary axis function in female patients with hepatolenticular degeneration (HLD). Method By RIA test the levels of serum pituitary and sex hormones were observed. Results The level of fallicle-stimulating hormone (FSH) was significantly lower than that in control group ( P <0 05),and the luteinizing hormone (LH),progesterone (P) and estradiol (E 2) levels were also significantly lower ( P <0 01), however,the patient’s sex hormone binding globulin (SHBG) was significantly higher than that of the control group ( P <0 001) A standard gonadotropin-releasing hormone(GnRH) test was performed in 10 of these patients, showing that most of them had a blunted LH and FSH responding to GnRH Conclusion The enzyme activity is reduced in cells of pituitary and gonad in HLD patients, which in turn reduces the endocrine function of glands. The hepatic copper sedimentation interferes with the metabolism of protein, causing the increase of the level of SHBG, and further reducing the physiologic availability of sex hormones