Citrin缺陷所致新生儿肝内胆汁淤积症的99Tcm-EHIDA肝胆动态显像

目的 研究citrin缺陷导致的新生儿肝内胆汁淤积症(NICCD)99Tcm-EHIDA肝胆动态显像特点及临床价值.方法 分别对NICCD组(12例)和NICCD阴性对照组(5例,4例婴儿肝炎综合征和1例脂类代谢异常)患儿年龄分别为(127±27)d和(164±15)d]进行99Tcm-EHIDA肝胆动态显像,观察注射显像剂后肝脏及肠道放射性分布,并采用秩和检验比较2组肝影持续时间及肠道显影时间.结果 NICCD组肠道显影时间和肝影持续时间均在180～1440min(中位数均为360min),而对照组肠道显影时间在15～30min(中位数为15min),肝影持续时间在60～180min(中位数为60min).NICCD患儿肝影持续时间及肠道显影时问均明显延长(Z=-3.20和-3.17,P均＜0.05).3例NICCD患儿肝显影不清晰,其中1例NICCD患儿胆囊及肠道在24h内始终未见显影,治疗后该患儿肝胆动态显像示肝摄取及排泄功能明显改善,15min肠道显影.结论 99Tcm-EHIDA肝胆动态显像示NICCD患儿肝脏摄取和排泄功能降低,提示99Tcm-EHIDA肝胆动态显像在NICCD诊断中可作为辅助检查手段.

^99Tc^m-EHIDA hepatobiliary imaging for neonatal intrahepatic cholestasis caused by citrin deficiency

Objective To evaluate the imaging characteristics of ^99Tc^m-EHIDA hepatobiliary scintig- raphy in neonatal intrahepatic cholestasis caused by citrin deficiency （NICCD）. Methods ^99Tc^m-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 between 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 rain （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 rain （ median = 15 rain） , respectively. The differences of hepatic activity retention time and bowel activity visualization time between the two groups were statistically signifi- cant （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 ^99Tc^m-EHIDA hepatobiliary imaging, which may be used in NICCD diagnosis and treatment response evaluation.