小儿胆道闭锁与肝内胆汁淤积症的鉴别诊断

Differential diagnosis of biliary atresia and intrahepatic cholestasis in children

目的探讨临床诊断方法对于鉴别诊断小儿胆道闭锁(BA)和肝内胆汁淤积症(IHC)的价值,从而提高临床医生对BA的认识,减少临床漏诊及误诊率。方法收集2011年7月-2014年6月以持续性黄疸收入中国医科大学附属盛京医院小儿消化内科的133例持续性黄疸患儿,分为IHC组111例,BA组22例。回顾性分析一般临床资料,并评价不同临床表现、实验室检查及影像学检查对BA和IHC的鉴别意义。计量资料组间比较采用t检验,计数资料组间比较采用χ2检验,不符合χ2检验条件时采用Fisher精确检验。结果临床表现中白便及肝脾增大伴有质地变硬在两组间差异具有统计学意义(χ2值分别为43.838、53.298、25.610,P值均<0.01);实验室检查BA组TBil、DBil、GGT、ALT、AST水平显著高于IHC组,差异均有统计学意义(t值分别为-5.628、-6.781、-7.485、-0.281、-4.349,P值均<0.01);GGT的特异度、阳性预测值、阳性似然比最高,而TBil具有最高的敏感度、阴性预测值以及最低的阴性似然比;TBil、DBil及GGT平行实验敏感度及阴性预测值可达到100%,三者系列实验特异度为98.0%,阳性预测值为88.9%。肝胆脾彩超探及肝门部纤维包块、胆囊收缩不良及磁共振胰胆管造影(MRCP)表现在两组间差异具有统计学意义(χ2值分别为106.635、16.433,P值均<0.01),系列实验特异度及阳性预测值均提高到100%。结论白便、肝脾大及质地变硬、TBil、DBil、GGT、肝胆彩超及MRCP表现对于BA及IHC诊断有鉴别意义,联合应用能提高诊断准确性。

Objective To investigate the significances of clinical diagnostic approaches to biliary atresia（ BA） and intrahepatic cholestasis（ IHC） in children,and to improve clinicians＇ understanding of BA and reduce the rates of missed diagnosis and false diagnosis. Methods A total of 133 children diagnosed with cholestasis with persistent jaundice admitted to our department from July 2011 to June 2014 were divided into IHC group with 111 patients and BA group with 22 patients. The general clinical trial data were reviewed and analysed and the significances of clinical manifestations,laboratory examination,and imaging features for differential diagnosis of BA and IHC were evaluated. Comparison of continuous data between the two groups was made by t test and comparison of categorical data between the two groups was made by chi- square test. When the sample characteristics for chi- square test were not suitable,the comparison was made by Fisher＇s test.Results Significant differences in clinical manifestations of kaolin stools and enlarged and hardened liver and spleen were observed between the two groups（ P 0. 01）. Total bilirubin（ TB）,direct bilirubin（ DB）,gamma GGT（ γ- GT）,alanine aminotransferase（ ALT）,and aspartate aminotransferase（ AST） levels in BA group were significantly higher than those in IHC group（ P 0. 01）. The specificity,positive predictive value,and positive likelihood ratio of γ- GT were the highest among them,while TB had the highest sensitivity,the highest negative predictive value,and the lowest negative likelihood ratio. The sensitivity and negative predictive value of TB,DB,and γ- GT in the parallel experiment reached 100%. The specificity and positive predictive value of them in the serial experiment were 98% and 88. 9%,respectively. There were significant differences in the hepatic portal fibrous mass and gallbladder hypokinesia detected with ultrasonography of the liver,gallbladder,and spleen and in magnetic resonance cholangiopancreatography（ MRCP） features between the two groups（ P 0. 01）. The specificity and positive predictive values of them in serial experiment reached 100%. Conclusion Kaolin stools,enlarged and hardened liver and spleen,TB,DB,γ- GT,ultrasonography,and MRCP of the liver and gallbladder are important indices to distinguish between BA and IHC and a combined analysis of them can improve the diagnostic accuracy.