Citrin缺陷所致婴儿肝内胆汁淤积症的肝胆显像特征

Imaging characteristics of hepatobiliary scintigraphy in neonatal intrahepatic cholestasis caused by citrin deficiency

目的 探讨citrin缺陷导致的婴儿肝内胆汁淤积症（NICCD）患儿^99Tc^m-二乙基乙酰苯胺亚氨二醋酸（EHIDA）肝胆显像的特征.方法 回顾性分析28例（男16例,女12例,1～8月龄）经基因确诊、同时行^99Tc^m-EHIDA肝胆动态显像检查的NICCD患儿资料.肝胆动态显像时心、肝、肾按正常时序和强度显影定义为摄取功能正常,肝影模糊和（或）心肾影持续时间延长者为摄取功能差;60 min内肠道显影为排泄通畅,60 min后为排泄延迟,24 h肠道仍不显影为排泄受阻.分析肝胆显像特征与血清总胆红素（TB）、直接胆红素（DB）、ALT、总胆汁酸（TBA）等指标间的关系.数据分析采用Kruskal-Wallis秩和检验.结果 28例中,20例表现为摄取功能正常,其中10例排泄通畅,10例排泄延迟;8例表现为摄取功能差,其中4例排泄延迟,4例排泄受阻.与摄取功能正常组相比,摄取功能差组TB和DB明显增高[183.6（128.7～ 280.9） mmol/L和105.5（80.0～ 141.7） mmol/L,135.6（95.7～212.6） mmol/L和73.1（53.9～ 97.9） mmol/L;Z=-2.25和-2.73,均P＜0.05].与排泄通畅者相比,排泄延迟者TB、DB和TBA明显升高[分别为137.5（122.0～170.9） mmol/L和81.7（65.7～93.5） mmol/L,96.5（81.1～ 108.0） mmol/L和54.1（45.3～72.6） mmol/L,245.6（183.9～299.2） mmol/L和136.0（73.5 ～163.2） mmol/L;Z=-3.92、-3.74和-2.57,均P＜0.05];排泄受阻者TB[262.0（152.1 ～ 542.8） mmol/L]和DB[192.7（118.1～407.2） mmol/L]更高（均Z=-2.82,均P＜0.05）.与排泄延迟组相比,排泄受阻者ALT明显增高[71.5（48.5～144.8） U/L和20.0（16.5～27.7） U/L;Z=-2.66,P＜0.05）.结论 ^99Tc^m-EHIDA肝胆显像可反映NICCD患儿肝脏摄取和排泄功能受损的状况,摄取功能受损严重时,可出现排泄受阻的征象.

Objective To investigate the scintigraphic features of neonatal intrahepatic cholestasis caused by citrin deficiency （NICCD） and to explore the clinical significance of ^99Tc^m-EHIDA hepatobiliary scintigraphy.Methods Hepatobiliary scintigraphy with ^99Tc^m-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 （TB：183.6（128.7-280.9） mmol/L vs 105.5（80.0-141.7） mmol/L,Z=-2.25;DB：135.6 （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 （TB：137.5 （122.0-170.9） mmol/L vs 81.7（65.7-93.5） mmol/L,Z=-3.92;DB：96.5 （81.1-108.0） mrrol/L vs 54.1（45.3-72.6） mmol/L,Z=-3.74; TBA：245.6（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 ^99Tc^m-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.