CAJ | 학술논문

目的：评价核素肝胆显像在评估citrin缺陷所致新生儿肝内胆汁淤积症（NICCD）患儿的肝脏摄取和排泄功能中的价值。方法应用单光子发射型计算机断层扫描仪（SPECT）对12例经SLC25A13基因诊断为NICCD的患儿进行核素肝胆动态显像，观察肝脏摄取和排泄功能，并与血生化检查结果进行比较分析。结果12例NICCD患儿在初诊时均有肝脏摄取功能严重受损和排泄功能障碍，其中5例在治疗随访中进行了第2次核素肝胆动态显像，显示肝脏摄取和排泄功能基本正常。NICCD患儿的肝脏摄取和排泄功能与肝细胞病变和胆汁淤积程度一致。结论核素肝胆显像对NICCD患儿肝脏摄取和排泄功能的评估具有重要价值。
목적：평개핵소간담현상재평고citrin결함소치신생인간내담즙어적증（NICCD）환인적간장섭취화배설공능중적개치。방법응용단광자발사형계산궤단층소묘의（SPECT）대12례경SLC25A13기인진단위NICCD적환인진행핵소간담동태현상，관찰간장섭취화배설공능，병여혈생화검사결과진행비교분석。결과12례NICCD환인재초진시균유간장섭취공능엄중수손화배설공능장애，기중5례재치료수방중진행료제2차핵소간담동태현상，현시간장섭취화배설공능기본정상。NICCD환인적간장섭취화배설공능여간세포병변화담즙어적정도일치。결론핵소간담현상대NICCD환인간장섭취화배설공능적평고구유중요개치。
Objectives To investigate the value of hepatobiliary scintigraphy in evaluation of the hepatic uptake and excre-tion function in neonatal intrahepatic cholestasis caused by citrin deifciency (NICCD). Methods Hepatobiliary scintigraphy with SPECT was used to detect the hepatic uptake and excretion function of 12 patients with NICCD conifrmed by SLC25A13 gene analysis, and the results were compared with the results of blood biochemical tests. Results The hepatic uptake and excretion function were obviously impaired in all of 12 NICCD patients in the initial scintigraphy. The scintigraphy were performed again in 5 patients in the follow-up after treatment, and showed that the hepatic uptake and excretion function was recovered. It was sug-gested that the hepatic uptake and excretion function was consistent with the level of liver enzymes and the degree of cholestasis. Conclusions Hepatobiliary scintigraphy is of value in evaluation of the hepatic uptake and excretion function in NICCD patients.