目的 探讨临床生化指标、瞬时弹性成像技术及无创肝纤维化评分在肝豆状核变性（WD）儿童进展期肝纤维化诊断中的价值。方法 收集2018年10月至2021年7月22例WD患儿的临床资料。患儿均行肝脏穿刺,采用瞬时弹性成像技术检测肝脏硬度值（LSM）。根据病理检查结果将患儿分为进展期肝纤维化（S3和S4期）和非进展期肝纤维化（S0、S1和S2期）两组,计算3种无创肝纤维化评分（Sheth、APRI和FIB-4指数）,比较两组间临床生化指标、无创肝纤维化评分和LSM的差异。采用受试者工作特征（ROC）曲线分析各指标对进展期肝纤维化的诊断价值。结果 22例WD患儿中,男12例、女10例,平均年龄（8.6±2.7）岁;非进展期肝纤维化为13例（59.1%）,进展期肝纤维化9例（40.9%）。与非进展期肝纤维化组相比,进展期肝纤维化组中白蛋白、天冬氨酸氨基转移酶（AST）和血小板（PLT）水平较低,凝血酶原时间（PT）和国际标准化比值（INR）水平较高,LSM、Sheth和FIB-4指数较高,差异均有统计学意义（P均<0.05）。ROC曲线分析提示,LSM（AUC=1.0）对进展期肝纤维化的诊断效能最大,其次为AST、PLT、PT、INR、FIB-4和Sheth指数（AUC均>0.7）。结论 AST、PLT、PT、INR、LSM、Sheth和FIB-4指数在儿童WD进展期肝纤维化中有一定的诊断价值,可以作为进展期肝纤维化的无创诊断参考指标。

Objective To investigate the value of clinical biochemical parameters, transient elastography and noninvasive liver fibrosis score in the diagnosis of advanced liver fibrosis in children with Wilson's disease (WD). Methods The clinical data of 22 WD patients from October 2018 to July 2021 were collected. All the children underwent liver biopsy, and the liver stiffness measurement (LSM) was measured by transient elastography. According to the pathological examination results, the children were divided into advanced liver fibrosis (S3 and S4) group and non-advanced liver fibrosis (S0, S1 and S2) group. Three kinds of non-invasive liver fibrosis scores (Sheth, APRI and FIB-4 index) were calculated, and the differences of clinical biochemical indexes, non-invasive liver fibrosis score and LSM between the two groups were compared. Receiver operating characteristic (ROC) curve was used to analyze the diagnostic value of each index for advanced liver fibrosis. Results Twenty-two WD children (12 boys and 10 girls) were included, with an average age of (8.6±2.7) years. There were 13 cases (59.1%) with non-advanced liver fibrosis and 9 cases (40.9%) with advanced liver fibrosis. Compared with non-advanced liver fibrosis group, the levels of albumin, aspartate aminotransferase (AST) and platelet (PLT) in advanced liver fibrosis group were lower, the prothrombin time (PT) and international standardized ratio (INR) were higher, and the indexes of LSM, Sheth and FIB-4 were higher, and the differences were significant (P<0.05). ROC curve analysis indicated that LSM (AUC=1.0) had the highest diagnostic efficacy for advanced liver fibrosis, followed by AST, PLT, PT, INR, FIB-4 and Sheth index (AUC>0.7). Conclusions AST, PLT, PT, INR, LSM, Sheth and FIB-4 index have certain diagnostic values in children with WD advanced liver fibrosis, and they can be used as a noninvasive diagnostic reference index in advanced liver fibrosis.