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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 secretory function of hypothalamus pituitary ovarian axis in female patients with hepatolenticular degeneration (HLD). Methods Serum pituitary and sex hormone levels were measured by radioimmunoassay in 13 HLD patients (HLD group) and 15 normal controls. Results The follicle stimulating hormone (FSH) in HLD patients was significantly lower than that in the normal group (P <0 05), and the progesterone (P), luteinizing hormone (LH) and estradiol ), And sex hormone binding globulin (SHBG) were significantly higher than those in the normal control group (P <0.01). Ten patients underwent gonadotropin-releasing hormone (GnRH) test and found that the pituitary gland stimulation of exogenous GnRH slow. Conclusions The activity of some enzymes in pituitary gland and gonadal cell of HLD patients is decreased and the secretion of hormones is decreased. At the same time, copper deposition in the liver disrupts protein metabolism, increases SHBG and further reduces the biological effects of sex hormones.