Background  Neonatal intrahepatic cholestasis caused by Citrin deficiency and biliary atresia are two diseases with similar clinical symptoms, which are difficult to distinguish according to clinical manifestations. Therefore, the differential diagnosis of the two diseases is of great significance.

  Objective   To explore the differences in expression levels of total bilirubin (TBIL), γ -glutamyltransferase (GGT), alkaline phosphatase (ALP) and total protein (TP) between the two diseases.

  Methods   Databases including PubMed, Web of science, Embase, CNKI and Wanfang Data were searched systematically. The retrieval time was up to March 2021. The literature quality of the included studies was evaluated according to the NOS (Newcastle-Ottawa scale, NOS) scale. After data extraction, the meta-analysis was performed using Stata 16.0 software.

  Results   Five English and four Chinese literatures were included in this study from 2005 to 2019, with NOS scores ranging from 5-8. There were 189 cases of neonatal intrahepatic cholestasis (NICCD) caused by Citrin deficiency and 325 cases of biliary atresia (BA). Meta-analysis results showed that the levels of TBIL, GGT and TP in NICCD group were significantly lower than those in BA group (SMD=-0.41, 95%CI -0.75, -0.07, SMD=-1.03, 95% CI -1.23, -0.84, SMD=-1.69, 95%CI -1.99, -1.38, all P＜0.05), but the level of ALP was higher than that of BA group (SMD=1.37, 95%CI 0.52, 2.21, P＜0.05). The heterogeneity analysis of TBIL and ALP showed high heterogeneity (66.3%, 86.4%). Subgroup analysis based on the ratio of sample size indicated that sample size might be one of the sources of heterogeneity.

  Conclusion   The differences of TBil, GGT, ALP and TP in NICCD and BA are statistically significant, which can provide some references for the early clinical differential diagnosis of BA and NICCD.