76例儿童急性肝衰竭病因及临床转归分析

Analysis on etiology and clinical outcome of 76 children with acute liver failure

目的 分析儿童急性肝衰竭(ALF)的病因和临床转归。方法 收集2010年1月—2021年1月期间长江大学附属仙桃市第一人民医院收治ALF患儿76例(男43例、女33例),年龄1个月~12岁。定期随访,依据临床转归结果将患儿分为存活组、死亡组,搜集ALF患儿病因,比较存活组、死亡组临床资料并作多因素分析。结果 76例ALF患儿病因未明确31例(40.8%)、病因明确45例(59.2%)。后者药物源性损害14例(18.4%)、感染性疾病11例(15.8%)、解剖异常(血管或胆道先天发育异常)9例(14.5%)、遗传代谢性疾病7例(9.2%)、自身免疫性疾病3例(3.9%)及肿瘤性疾病1例(1.3%)。76例ALF患儿中存活、死亡分别为44例、32例,比较临床资料可知,存活组ALT、AST[1 108(504, 3 114)U/L、1 226(808, 3 225)U/L]均显著高于死亡组[526(204, 915)U/L、717(470, 1 948)U/L,Z=27.402, 24.153,P<0.05];存活组Alb、TBil、DBil及血氨分别为33.2(30.2, 38.4)g/L、61.2(26.5, 182.4)μmol/L、38.0(12.9, 114.8)μmol/L及80.0(60.5, 101.7)μmol/L,与死亡组[28.7(26.0, 31.4)g/L、152.9(70.6, 288.0)μmol/L、87.6(41.6, 158.2)μmol/L及109.5(85.5, 146.6)μmol/L]相比,差异具有统计学意义(Z=8.162,-30.429,-48.624及-9.489,P<0.05);存活组PT、INR[24.2(21.0, 29.8)s、2.1(1.8, 2.8)]均显著低于死亡组[38.4(24.4, 52.5)s、3.3(2.3, 6.6),Z=-20.197,-21.158,P<0.05];存活组PELD评分[20.2(18.4, 32.0)分]显著高于死亡组[30.8(24.6, 39.0)分,Z=-22.278,P<0.05]。将ALT、AST、Alb、TBil、DBil、血氨、PT、INR及PELD评分作为自变量,ALF患儿临床转归结局作为应变量(赋值0=存活,1=死亡),纳入logistic回归分析,结果提示TBil及PELD评分是ALF患儿临床转归中死亡发生的独立危险因素(P<0.05)。结论 儿童ALF病死率高,常见病因包括药物源性损害、感染性疾病、解剖异常(血管或胆道先天发育异常)、遗传代谢性疾病、自身免疫性疾病及肿瘤性疾病。ALF患儿TBil、PELD评分升高提示预后不良。

Objective To investigate the etiology and clinical outcome of 76 children with acute liver failure(ALF). Methods A total of 76 children(43 males and 33 females) with ALF admitted to our hospital from January 2010 to January 2021 were included, with an average age of 1 month to 12 years. All the objects were followed up regularly and death group according to the clinical outcome. The etiology of ALF children were collected, and the clinical data of survival group and death group were compared and analyzed by multiple-factor analysis. Results The etiology of 31(40.8%) cases was unclear and etiology of 45(59.2%) cases was definite. Among the latter, 14 cases were drug-induced injury(18.4%), 11 cases were infectious diseases(15.8%), 9 cases were anatomical abnormalities(congenital dysplasia of blood vessels or biliary tract)(14.5%), 7 cases were hereditary metabolic diseases(9.2%), 3 cases were autoimmune diseases(3.9%) and 1 case was neoplastic diseases(1.3%). Among the 76 children with ALF, 44 cases survived and 32 cases died, respectively. Compared with clinical data, the alanine transaminase(ALT) and aspartate aminotransferase(AST) [1108(504, 3114) U/L and 1226(808, 3225) U/L] in the survival group were significantly higher than those in the death group [526(204, 915) U/L and 717(470,1948) U/L, Z=27.402, 24.153, P<0.05]. In the survival group, the albumin(Alb), total bilirubin(TBil), direct bilirubin(DBil) and blood ammonia of survival group [33.2(30.2, 38.4) g/L, 61.2(26.5, 182.4) μmol/L, 38.0(12.9, 114.8) μmol/L and 80.0(60.5, 101.7) μmol/L] were significantly different from those in death group [28.7(26.0, 31.4) g/L、152.9(70.6, 288.0) μmol/L, 87.6(41.6, 158.2) μmol/L and 109.5(85.5, 146.6) μmol/L], P<0.05. The survival group plotting time PT and international normalized ratio(INR) were 24.2(21.0, 29.8) s and 2.1(1.8, 2.8), which was significantly different from the death group [38.4(24.4, 52.5) s and 3.3(2.3, 6.6)](Z=-20.197,-21.158, P<0.05). PELD score of survival group [20.2(18.4, 32.0) points] was significantly higher than that of death group [30.8(24.6, 39.0) points, Z=-22.278, P<0.05]. Taking the ALT, AST, Alb, TBil, DBil, blood ammonia, PT, INR and PELD score as independent variables, and the clinical outcome was taken as dependent variable(assigned 0 = survival, 1 = death), and the logistic regression analysis was conducted. The results indicated that TBil and PELD scores were independent risk factors of death in the clinical outcome of children with ALF(P<0.05). Conclusion The mortality of children with ALF is high, and the common causes are drug-induced damage, infectious diseases, anatomical abnormalities(congenital dysplasia of blood vessels or biliary tract), genetic metabolic diseases, autoimmune diseases and neoplastic diseases. The increase of TBil and PELD scores in children with ALF indicates poor prognosis.