Objective To investigate the expression and the possible clinical significance of serum Golgi protein(GP73) in infantile hepatitis syndrome(IHS) by different causes. Methods Totally 79 patients with IHS in Guangzhou Women and Children′s Medical Center from February 2012 to December 2012 were enrolled in this study, including 15 cases with biliary atresia(BA) group, 29 cases with infection(infection group), 5 cases with neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD group), and 30 cases with unknown etiology(idiopathic infantile hepatitis group). At the same time, 30 healthy infants were enrolled as healthy control group.The serum levels of GP73 were determined by quantitative enzyme-linked immunosorbent assay(ELISA), and the children′s liver function[alanine aminotransferase(ALT), aspartate aminotransferase(AST), total bilirubin(TBIL), direct bilirubin(DBIL), alkaline phosphatase(ALP), γ-glutamyl trans-peptidase(γ-GT), total bile acid(TBA) and albumin(ALB)] were measured by turbidimetric inhibition immuno assay.Then, the corresponding data were statistically analyzed. Results Serum GP73 in BA group, infection group, NICCD group, idiopathic infantile hepatitis group and the healthy control group were (296.6±67.5) μg/L, (185.1±66.4) μg/L, (199.2±87.1) μg/L, (181.7±74.2) μg/L and (65.3±17.0) μg/L, respectively.Serum γ-GT levels in BA group, infection group, NICCD group, idiopathic infantile hepatitis group and healthy control group were (764.7±775.8) U/L, (448.2±352.7) U/L, (239.4±88.7) U/L, (283.3±377.2) U/L and (54.0±72.6) U/L, respectively.The levels of GP73 and γ-GT were significantly higher in infants with IHS, and the levels of GP73 and γ-GT in infants with BA were the highest(F=46.775, 9.238, all P 0.05). The receiver operating characteristic curve(ROC) constructed with GP73 showed a sensitivity of 80.0% and specificity of 82.8% with an area under the receiver(AUC) of 0.872 for diagnosis of BA, comparatively, a sensitivity of 66.7% and specificity of 71.9% were showed with a AUC of 0.731 when performed with γ-GT. Conclusions Serum GP73 concentration significantly increased in all liver disease groups, regardless of the etiology.Serum GP73 expression is significantly higher in infants with BA.Serum GP73 shows a superior sensitivity and specificity to γ-GT for diagnosis of BA, which might be useful for early diagnosis of BA and IHS with different causes. Key words: Infantile hepatitis syndrome; Golgi protein 73; Biliary atresia