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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。
Objective: To investigate the diagnostic value of serological markers of liver fibrosis in sibling siblings with no evidence of hepatolenticular lesions in patients with hepatolenticular degeneration (HLD). Methods: 94 siblings diagnosed as HLD without hepatolenticular lesions were selected as the HLD group from the siblings diagnosed as having HLD. Among the 70 HLD families, siblings who had no diagnosis of HLD lesions were examined to exclude HLD from being non-HLD Group; 70 healthy volunteers as healthy control group. The serum levels of hyaluronic acid (HA), laminin (LN) and collagen Ⅳ (Ⅳ-c) in liver fibrosis were detected and analyzed by radioimmunoassay. Results: The average values of HA, LN and Ⅳ-C in HLD group were (139.26 ± 74.15) ng · mL -1, (126.85 ± 24.44) ng · mL -1 and (82.02 ± 31.23) ng · mL, significantly higher than those in non-HLD group (51.32 ± 19.54 ng · mL -1, 65.72 ± 20.35 ng · mL -1, 34.82 ± 11.56 ng · mL -1, (-1) and healthy controls (46.75 ± 20.93 ng · mL -1, 68.92 ± 22.17 ng · mL -1 and 33.57 ± 12.39 ng · mL -1, respectively) P <0.01 respectively. There was no significant difference between non-HLD group and healthy control group (P> 0.05). Conclusion: Serological detection of liver fibrosis can be used as one of the indicators of sibling screening and clinical diagnosis of HLD in HLD patients without HLD lesions.