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目的分析Citrin蛋白缺陷所致新生儿肝内胆汁淤积症(NICCD)患儿的胆汁成分,探讨NICCD患儿肝内胆汁淤积的胆汁成分变化。方法 2010年3月至2012年10月应用婴儿十二指肠引流管收集武汉华中科技大学同济医学院附属同济医院儿童感染消化科经SLC25A13基因突变诊断的12例NICCD患儿胆汁,采用德国罗氏MODULAR生化检测仪定量检测胆汁中总胆红素、直接胆红素、总胆汁酸、胆固醇值,以8例无肝胆疾病婴儿胆汁成分作为对照组进行分析。结果 12例NICCD患儿胆汁中总胆红素、直接胆红素、总胆汁酸、胆固醇值显著低于对照组,差异有统计学意义(P均<0.05)。结论 NICCD患儿肝脏分泌和排泄胆红素、胆汁酸、胆固醇功能下降,其严重程度与肝功能损伤程度一致。
OBJECTIVE: To analyze the biliary components in neonates with intrahepatic cholestasis of neonate (NICCD) caused by Citrin deficiency and to explore the changes of bile components in intrahepatic cholestasis of children with NICCD. Methods From March 2010 to October 2012, 12 cases of NICCD patients diagnosed as SLC25A13 gene mutation in Tongji Hospital of Tongji Medical College of Tongji Medical College, Huazhong University of Science and Technology from March 2010 to October 2012 were collected by using duodenal drainage tube. German Roche MODULAR Biochemical analyzer quantitative detection of bile in total bilirubin, direct bilirubin, total bile acid, cholesterol value, with 8 cases of non-liver and gallbladder infant bile composition as a control group analysis. Results The total bilirubin, direct bilirubin, total bile acid and cholesterol in 12 NICCD children were significantly lower than those in control group (P <0.05). Conclusions The liver of NICCD patients secrete and excretion of bilirubin, the function of bile acid and cholesterol are decreased, and their severity is consistent with the degree of liver damage.