肝纤维化血清学指标对肝豆状核变性家族中无肝豆状核病变诊断依据的同胞兄妹的临床诊断意义  

Significance of Hepatic Fibrosis Serum Index Sign for Diagnosing Sibling of Hepatolenticular Degeneration Probands

作　　者：丛时兵[1] 吕有奎[1] 吴君霞[1] 洪铭范[1] 韩咏竹[1] 杨任民[1] 

机构地区：[1]安徽中医学院神经病学研究所,230031

出　　处：《中国临床神经科学》2008年第4期357-360,共4页Chinese Journal of Clinical Neurosciences

基　　金：安徽省卫生厅自然科学基金项目(项目号:2002A020)

摘　　要：目的:探讨肝纤维化血清学指标对肝豆状核变性(HLD)家族中无肝豆状核病变诊断依据的同胞兄妹的诊断意义。方法:选取94例HLD家族中无肝豆状核病变诊断依据的同胞兄妹经检查确诊为HLD者为HLD组;70例HLD家族中无HLD病变诊断依据的同胞兄妹经检查排除HLD者为非HLD组;70名健康自愿者为健康对照组。对3组肝纤维化血清学指标透明质酸(HA)、层黏连蛋白(LN)及Ⅳ型胶原(Ⅳ-c)用放射免疫法进行检测和分析。结果:HLD组HA、LN和Ⅳ- C平均值分别为(139.26±74.15)ng·mL^(-1)、(126.85±24.44)ng·mL^(-1)和(82.02±31.23)ng·mL^(-1);明显高于非HLD组(51.32±19.54)ng·mL^(-1)、(65.72±20.35)ng·mL^(-1)、(34.82±11.56)ng·mL^(-1)及健康对照组(46.75±20.93)ng·mL^(-1)、(68.92±22.17)ng·mL^(-1)和(33.57±12.39)ng·mL^(-1),P均<0.01;非HLD组与健康对照组间差异无统计学意义,P均>0.05。结论:肝纤维化血清学指标检测可作为HLD家族中无HLD病变诊断依据的同胞兄妹筛查和临床诊断HLD的指标之一。Study the significance of hepatic fibrosis serum index sign for diagnosing sibling of hepatolenticular degeneration（HLD） proband. Methods： Select at random 94 cases of HLD proband sibling as HLD group, who have been diagnosed with HLD and without showing any symptom. For the first time they are admitted into the hospital due to this diagnosis, not treated with decopper. 70 cases of sibling of HLD proband, who have been diagnosed without HLD, are used as the control group of non-HLD patients. 70 healthy persons are used as the healthy control group. Measure the hepatic fibrosis serum index sign of different groups of persons mentioned above, including HA, LN and Ⅳ -C with RIA and analyze them. Results. The average values of HA, LN and Ⅳ -C of the HLD group are respectively （139.26±74.15） ng·mL^-1, （126.85±24.44） ng.mL-1, （82.02±31.23）ng·mL^-1; which are obviously higher than those of the non-HLD group [（51.32±19.54）ng·mL^-1, （65.72±20.35）ng·mL^-1, （34.82±11.56）ng·mL^-1 as well as those of the healthy control group [（46.75±20.93）ng·mL^-1, （68.92±22.17）ng·mL^-1, （33.57±12.39） ng·mL^-1. Their P values are all less than 0.01. The differences between the non-HLD group and the healthy control group are not significant （P〉0.05）. Conclusion：Hepatic fibrosis serum index sign can be used as one of the indexes for screening and diagnosing HLD of siblings of HLD probands.

关 键 词：肝豆状核变性 透明质酸 层黏连蛋白 Ⅳ型胶原 筛查 诊断 

分 类 号：R742.4[医药卫生—神经病学与精神病学]