儿童急性肝衰竭120例病因和预后分析

Analysis of etiology and prognosis of 120 children with pediatric acute liver failure

目的探讨儿童急性肝衰竭(PALF)的病因、预后及预后影响因素,为临床治疗提供依据。方法收集2008年5月至2018年5月在湖南省儿童医院住院并确诊为PALF患儿的临床资料,对其病因和预后进行分析,并根据预后分为死亡组和存活组,比较2组生化指标,根据不同资料分别采用t检验、Wilcoxon检验和χ2检验进行统计学分析。结果120例PALF患儿中,男68例,女52例;婴儿36例,幼儿34例,学龄前期22例,学龄期28例。病因方面:脓毒症20例(16.7%)、遗传代谢性疾病19例(15.8%)、中毒18例(15.0%)、病毒感染12例(10.0%)、药物6例(5.0%)、胆道息肉1例(0.8%)、肿瘤性疾病1例(0.8%)和病因不明43例(35.9%)。在已知病因中,婴儿以感染和遗传代谢性疾病为主,幼儿以感染和药物/毒物为主,而学龄期和学龄前期以药物/毒物和遗传代谢性疾病为主;PALF患儿病死率为50.0%;EB病毒相关噬血细胞综合征、脓毒症、希特林蛋白缺乏症和酪氨酸血症合并PALF病死率高;与存活组比较,死亡组总胆红素(TB)[159.00(73.05,274.00)μmol/L比62.75(2.65,221.75)μmol/L]、结合胆红素(DB)[83.00(41.43,160.00)μmol/L比38.74(10.98,128.75)μmol/L]、凝血酶原时间(PT)[39.60(24.93,62.60)s比24.65(21.43,29.83)s]、国际标准化比值(INR)[3.40(2.30,6.74)比2.09(1.85,2.84)]、血氨(NH3)水平[109.50(85.25,149.75)μmol/L比80.00(60.25,102.75)μmol/L]升高,差异均有统计学意义(均P<0.05);死亡组清蛋白(ALB)[(28.72±5.88)g/L比(33.69±4.96)g/L]、丙氨酸转氨酶(ALT)[586.50(223.25,1082.00)U/L比1434.00(615.00,3334.50)U/L]和天冬氨酸氨基转移酶(AST)[827.50(545.00,2024.00)U/L比1663.50(821.00,4886.75)U/L]水平降低,差异均有统计学意义(均P<0.05),而血糖和胆固醇水平无明显变化。结论PALF病死率高,不同年龄段病因不同,TB、DB、PT、INR、NH3水平升高及肝性脑病发病率升高,ALB、ALT和AST降低提示预后不良。

Objective To investigate the etiology,prognosis and prognostic factors of pediatric acute liver failure(PALF),in order to provide the basis for clinical treatment of PALF.Methods The clinical data of children with PALF hospitalized at Hunan Children′s Hospital from May 2008 to May 2018 were collected,and the causes and prognosis were analyzed.According to the prognosis,the patients were divided into the death group and the survival group,whose biochemical indexes were then compared.After that,the statistical analysis of different data were carried out by using t-test,Wilcoxon test andχ2 test separately.Results In 120 PALF cases,there were 68 males and 52 females,and there were 36 infants,34 toddlers,22 preschoolers and 28 school-age children.Twenty cases(16.7%)were caused by sepsis,19 cases(15.8%)by genetic metabolic diseases,18 cases(15.0%)by poisoning,12 cases(10.0%)by viral infection,6 cases(5.0%)by drugs,1 case(0.8%)by bile polyp,and 1 case(0.8%)by tumor disease.Besides,the etiology of 43 cases(35.9%)was unknown.Among the cases with known etiologies,genetic metabolic and infectious diseases were the main cause of disease in infants,toddler patients were mostly caused by infectious diseases and drug/toxicants,and drug/toxicants and hereditary metabolic diseases were the dominant cause of disease in school-age children and preschoolers.Mortality rate of children with PALF was 50.0%.Among them,the mortality of Epstein-Barr virus-associated hemophagocytic syndrome,sepsis,Citrin deficiency and Tyrosinemia was higher than that of other diseases.Compared with the survival group,the total bilirubin(TB)[159.00(73.05,274.00)μmol/L vs.62.75(2.65,221.75)μmol/L],direct bilirubin(DB)[83.00(41.43,160.00)μmol/L vs.38.74(10.98,128.75)μmol/L],prothrombin time(PT)[39.60(24.93,62.60)s vs.24.65(21.43,29.83)s],international standardized ratio(INR)[3.40(2.30,6.74)vs.2.09(1.85,2.84)],and blood ammonia(NH3)levels[109.50(85.25,149.75)μmol/L vs.80.00(60.25,102.75)μmol/L]in the death group were significantly increased,and the diffe-rences were statistically significant(all P<0.05);while the levels of albumin[(28.72±5.88)g/L vs.(33.69±4.96)g/L],alanine aminotransferase(ALT)[586.50(223.25,1082.00)U/L vs.1434.00(615.00,3334.50)U/L]and aspartate aminotransferase(AST)[827.50(545.00,2024.00)U/L vs.1663.50(821.00,4886.75)U/L]in the death group were significantly decreased,and the differences were statistically significant(all P<0.05).However,the blood glucose and cholesterol levels in both groups had no statistically significant difference.Conclusion The mortality of children with PALF is high,and different age groups have different etiologies.The increase of TB,DB,PT,INR,NH3 and the ratio of hepatic encephalopathy,and the decrease of albumin,AST and ALT suggest poor prognosis.