肝纤维化指标的水平对肝豆状核变性的评估  

Assessment of hepatic fibrosis index in Wilson disease

作　　者：邵新华[1] 刘晓青[1] 徐芹芳[1] 吴洁[1] 乌玉丽[1] 张雅芬[1] 

机构地区：[1]上海交通大学医学院附属新华医院,上海市儿科医学研究所,200092

出　　处：《实用医学杂志》2010年第20期3701-3703,共3页The Journal of Practical Medicine

基　　金：国家教育部<高等学校骨干教师资助计划>资助项目(2000年度)

摘　　要：目的:探讨血清层粘蛋白(LN)、Ⅲ型前胶原(PCⅢ)、Ⅳ型胶原(C-Ⅳ)联合检测水平在肝豆状核变性(WD)纤维化发生、发展及治疗过程中的临床意义。方法:利用酶联免疫(ELISA)测定38例肝豆状核变性患者。其中男18例,女20例。起病年龄为4～39岁。临床表现为肝脏损害症状23例,神经症状8例,肝脏伴其他症状7例。初诊21例,治疗5年以上12例,不规则治疗5例。结果:本组血清肝纤维化指标水平对WD临床类型中无显著意义。治疗组、未治疗和不规则治疗组检测结果显示3组间血清LN和C-Ⅳ有显著意义。3组WD患者分别做3项指标检出率,经χ2检验,初诊的LN和C-Ⅳ明显高于经治疗组。结论:血清LN、PCⅢ及C-Ⅳ水平对评估WD的治疗和预后有参考价值。Objective To explore clinical significance of observed hepatic fibrosis index （Laminin,type Ⅲ procollagen and type Ⅳ collagen） during the course of clinical development and treatment in Wilson disease（WD）.Methods Contents of serum LN,PCⅢ and C-Ⅳ were detected by ELISA in 38 patients with Wilson disease enrolled in this study,18 males and 20 females.The age of onset ranged from 4 to 39 years old.Twenty-three of 38 patients had presented with hepatic manifestations,seven patients had hepatic and neurological manifestations or other symptoms at the same time,eight had only neurological symptom.21 patients were new patients,12 patients were in treatment above 5 years,5 patients were in anomalous-treatment.Results In this study,the differences in the contents of three liver fibrosis in clinical type groups were not significant （P 0.05）.There were remarkable differences among the new patients,anomalous-treatment and by the treatment 5 years above （P 0.05）.The positive rates of LN and C-Ⅳ levels were markedly higher in new WD patients than that of treatment group.Conclusion Combined determination of liver fibrosis may help the estimate of therapeutic efficacy and prognosis in WD patients.

关 键 词：肝豆状核变性 层粘蛋白 Ⅲ型前胶原 Ⅳ型胶原 

分 类 号：R575.2[医药卫生—消化系统]