摘要目的:通过回顾性分析胆汁淤积症患儿的临床资料，探讨γ-谷氨酰转肽酶(gamma-glutamyl transpeptidase，GGT)与肝杨氏模量在胆道闭锁(biliary atresia，BA)和婴儿肝内胆汁淤积(intrahepatic cholestasis，IHC)鉴别诊断中的应用价值。方法:收集2017年9月至2019年12月西安交通大学附属儿童医院住院治疗的胆汁淤积症患儿120例为研究对象，根据术中胆管造影结果和随访情况，将患儿分为BA组(50例)和IHC组(70例)。比较两组患儿就诊年龄、临床表现、实验室检查结果、胆囊收缩率、同位素肝胆显像、肝杨氏模量、内科治疗效果等指标，分析GGT与肝杨氏模量在BA与IHC鉴别诊断中的应用价值。结果:两组患儿的就诊年龄、丙氨酸氨基转移酶、天冬氨酸氨基转移酶、总胆汁酸、空腹血糖、血氨、脾大，差异无统计学意义( P>0.05)。两组患儿的大便颜色、肝脏大小、总胆红素(total bilirubin，TB)、直接胆红素(direct bilirubin，DB)、GGT、肝杨氏模量、同位素肝胆显像阳性、餐后1 h胆囊收缩率、内科治疗效果差异有统计学意义( P<0.001)。对TB、DB、GGT、肝杨氏模量和GGT＋肝杨氏模量进行ROC曲线分析，曲线下面积分别为0.820、0.809、0.906、0.876和0.926。当GGT超过界值198.85 U/L时提示患儿为BA的敏感度、特异度、阳性预测值、阴性预测值和准确度分别为82.0%、84.3%、78.8%、86.8%和83.3%。当肝杨氏模量超过界值8.6 kPa时提示患儿为BA的敏感度、特异度、阳性预测值、阴性预测值和准确度分别为88.0%、80.0%、75.9%、90.3%和83.3%。GGT与肝杨氏模量联合诊断提示患儿为BA的敏感度、特异度、阳性预测值、阴性预测值和准确度分别为98.0%、68.6%、69.0%、98.0%和80.8%。多因素Logistic回归分析发现，DB>115.55 μmol/L、GGT>198.85 U/L和肝杨氏模量>8.6 kPa均为BA的危险因素( OR＝9.510， P＝0.001； OR＝24.634， P<0.001； OR＝21.469， P<0.001)。 结论:GGT与肝杨氏模量在BA与IHC的鉴别诊断中有较好的应用价值，GGT超过界值198.85 U/L、肝杨氏模量超过界值8.6 kPa时能有效提示患儿为BA。

abstractsObjective:In this study, the clinical data of biliary atresia(BA) and infant intrahepatic cholestasis(IHC) was reviewed, and the utility of gamma-glutamyl transpeptidase(GGT) and liver Young′s modulus in the differential diagnosis of BA and IHC in infants was discussed.Methods:Based on the clinical data of 120 infants with cholestasis treated in the Children′s Hospital Affiliated to Xi′an Jiaotong University, from September 2017 to December 2019, the infants were divided into two groups according to the results of intraoperative cholangiography and follow-up: BA group( n=50); IHC group( n=70). The age, clinical manifestations, laboratory examination results, gallbladder contraction rate, hepatobiliary scintigraphy, liver Young′s modulus, and medical treatment effects were compared between the two groups.The utility of GGT and liver Young′s modulus in the differential diagnosis of BA and IHC was analyzed. Results:The age, alanine aminotransferase(ALT), aspartate aminotransferase(AST), total bile acid, fasting blood glucose, blood ammonia and splenomegaly between the two groups were compared and the results showed no statistical significance( P>0.05). In contrast, there were statistically significant differences( P<0.001) in stool color, liver size, total bilirubin(TB), direct bilirubin(DB), GGT, liver Young′s modulus, positive hepatobiliary scintigraphy, gallbladder contraction rate at 1 hour after meal, and medical treatment effect between the two groups.TB, DB, GGT, liver Young′s modulus and GGT combined with liver Young′s modulus were analyzed using ROC curves, and the area under the curve(AUC) were 0.820, 0.809, 0.906, 0.876 and 0.926, respectively.When GGT exceeded the cut-off value of 198.85 U/L, the sensitivity, specificity, positive predictive value, negative predictive value and accuracy of BA were 82.0%, 84.3%, 78.8%, 86.8% and 83.3%, respectively.When liver Young′s modulus exceeded the cut-off value of 8.6 kPa, the sensitivity, specificity, positive predictive value, negative predictive value and accuracy of BA were 88.0%, 80.0%, 75.9%, 90.3% and 83.3%, respectively.The sensitivity, specificity, positive predictive value, negative predictive value and accuracy of GGT combined with liver Young′s modulus in diagnosing BA were 98.0%, 68.6%, 69.0%, 98.0% and 80.8%, respectively.Multivariate logistic regression analysis found that DB>115.55 μmol/L, GGT>198.85 U/L, and liver Young′s modulus>8.6 kPa were risk factors for BA( OR=9.510, P=0.001; OR=24.634, P<0.001; OR=21.469, P<0.001). Conclusion:GGT and liver Young′s modulus are useful in the differential diagnosis of BA and IHC.If GGT and liver Young′s modulu sexceed the threshold values of 198.85 U/L and 8.6 kPa respectively, it can effectively indicate that the child is BA.