Imaging characteristics of hepatobiliary scintigraphy in neonatal intrahepatic cholestasis caused by citrin deficiency / 中华核医学与分子影像杂志

ABSTRACT

Objective To investigate the scintigraphic features of neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD) and to explore the clinical significance of 99Tcm-EHIDA hepatobiliary scintigraphy.Methods Hepatobiliary scintigraphy with 99Tcm-EHIDA was performed in 28 genetic confirmed NICCD cases (16 males,12 females,1-8 months).Normal dynamics (i.e.,time-activity curve) of the tracer in heart,liver and kidneys was defined as normal hepatic uptake function.Decreased liver uptake of the tracer and/or prolonged heart and kidneys radioactivity retention was defined as impaired hepatic uptake function.Normal or delayed biliary excretion was defined as radioactivity appearing in the bowel within 60 min or after 60 min.Occluded biliary excretion was defined as absent bowel radioactivity within 24 h.The characteristics of hepatobiliary scintigraphy and their relationship with levels of serum total bilirubin (TB),direct bilirubin(DB),ALT,total bile acid (TBA) were retrospectively analyzed.Statistical analysis was performed using Kruskal-Wallis rank sum test.Results Of 28 NICCD patients,20 showed normal uptake while 8 had impaired hepatic uptake.Ten of twenty patients with normal uptake function showed normal biliary excretion and the others showed delayed biliary excretion.Four of eight cases who presented impaired hepatic uptake function were with delayed biliary and the rest displayed biliary excretion occlusion.Compared with the patients with normal hepatic uptake function,patients with impaired hepatic uptake had significantly higher levels of TB and DB (TB183.6(128.7-280.9) mmol/L vs 105.5(80.0-141.7) mmol/L,Z=-2.25;DB135.6 (95.7-212.6) mmol/L vs 73.1(53.9-97.9) mmol/L,Z=-2.73; both P＜0.05).Compared with the cases with normal biliary excretion function,patients with delayed biliary excretion had significantly higher levels of TB,DB and TBA (TB137.5 (122.0-170.9) mmol/L vs 81.7(65.7-93.5) mmol/L,Z=-3.92;DB96.5 (81.1-108.0) mrrol/L vs 54.1(45.3-72.6) mmol/L,Z=-3.74; TBA245.6(183.9-299.2) mmol/L vs 136.0(73.5-163.2) mmol/L,Z=-2.57; all P＜0.05).The levels of TB (262.0(152.1-542.8) mmol/L) and DB (192.7(118.1-407.2) mmol/L; both Z=-2.82; both P＜0.05) were the highest in patients with occluded biliary excretion.Compared with the patients with delayed excretion,the occluded excretion subgroup had significantly higher levels of ALT (71.5 (48.5-144.8) U/L vs 20.0(16.5-27.7) U/L,Z=-2.66,P＜0.05).Conclusion 99Tcm-EHIDA hepatobiliary scintigraphy may evaluate hepatic uptake and excretion function of the NICCD infants.When the hepatic uptake is remarkably decreased,the occluded biliary excretion can be shown.