Abstract： Objective To evaluate the imaging characteristics of 99Tcm-EHIDA hepatobiliary scintigraphy in neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD).Methods 99Tcm-EHIDA hepatobiliary scan was performed on 12 NICCD infants (aged (127 ±27) d) and 5 infants as a control group (including 4 cases with hepatitis syndrome and 1 with abnormal lipid metabolism,aged (164 ± 15) d).The differences of hepatic activity retention time and bowel activity visualization time bctwccn the two groups were observed.The two-sample Wilcoxon rank sum test was used to analyze the data.Results In the NICCD group,both hepatic activity retention time and bowel activity visualization time were 180-1440 min (median =360 min).In the control group,hepatic activity retention time and bowel activity visualization time were 60-180 min (median =60 min) and 15-30 min (median =15 min),respectively.The differences of hepatic activity retention time and bowel activity visualization time between the two groups were statistically significant (Z =-3.20 and-3.17,both P ＜ 0.05).Three NICCD infants showed minimal hepatic uptake of the tracer.The bowel activity was not visible in 1 NICCD case.The hepatic uptake and biliary excretion function of this infant were significantly improved on hepatobiliary scintigraphy after treatment,with a bowel activity visualization time of 15 min.Conclusion NICCD infants show impaired hepatic uptake and biliary excretion function on 99Tcm-EHIDA hepatobiliary imaging,which may be used in NICCD diagnosis and treatment response evaluation.