山东省多中心PICU收治的急性肝衰竭患儿病因、临床特点及预后影响因素  

Etiology,clinical characteristics and prognostic factors of children with pediatric acute liver failure admitted to PICU in Shandong Province:a multi-center retrospective study

作　　者：王琦玮 杨鹏 韩明英[3] 侯建[4] 赵春 王伟 王玉娟 信晓伟 靳有鹏 WANG Qiwei;YANG Peng;HAN Mingying;HOU Jian;ZHAO Chun;WANG Wei;WANG Yujuan;XIN Xiao-wei;JIN Youpeng(不详;Department of Pediatric Intensive Care Medicine,Shandong Provincial Hospital Affiliated to Shandong First Medical University,Jinan 250021,China)

机构地区：[1]山东第一医科大学附属省立医院小儿重症医学科,济南250021 [2]山东第一医科大学第二附属医院小儿重症医学科 [3]临沂市人民医院小儿重症医学科 [4]淄博市妇幼保健院小儿重症医学科

出　　处：《山东医药》2023年第18期1-5,共5页Shandong Medical Journal

基　　金：中国初级卫生保健基金会临床应用研究专项(MTP2022D029);泰山学者工程专项经费资助项目;济南市临床医学科技创新计划(202019177)。

摘　　要：目的总结急性肝衰竭(pediatric acute liver failure,PALF)患儿的病因、临床特点及预后影响因素。方法对2010年8月—2022年8月间山东省4家儿童重症监护病房(PICU)收治的236例急性肝衰竭患儿的临床资料作回顾性分析。结果236例患儿病因有感染性疾病49例(20.8%)、遗传代谢性疾病36例(15.3%)、噬血细胞综合征27例、中毒19例、恶性肿瘤/血液病12例、循环衰竭11例,胆道疾病3例、热射病1例及不明原因78例(33.0%)。236例患儿中男性131例(55.5%);年龄0~1岁109例(46.2%);临床表现为黄疸160例(67.8%)、多器官功能障碍综合征(MODS)148例、出血118例、腹水80例、肝性脑病75例、DIC 67例、肝肾综合征20例。236例患儿治疗后好转出院87例、治疗无效149例。凝血酶原时间(PT)延长、血氨(NH3)水平升高、血清白蛋白(ALB)水平降低及合并MODS是急性肝衰竭患儿预后不良的独立危险因素(OR分别为1.023,1.008,0.928,2.980;95%CI分别为1.003~1.044,1.002~1.014,0.872~0.989,1.393~6.377;P均<0.05)。结论急性肝衰竭患儿的最常见病因是感染性疾病。急性肝衰竭多发生于1岁以下男婴,主要临床表现为黄疸、MODS及出血,预后较差,PT延长、血NH3水平高、血清ALB水平低及合并MODS的急性肝衰竭患儿预后更差。Objective To investigate the etiology,clinical characteristics,and prognostic factors of pediatric acute liver failure(PALF)in children.Methods The clinical data of 236 children with PALF who were admitted to four PICUs in Shandong Province between August 2010 and August 2022 were retrospectively reviewed.Results A total of 236 patients with PALF were enrolled,including 49 cases of infectious diseases(20.8%),36 cases of genetic metabolic diseases(15.3%),27 cases of hemophagocytic syndrome,19 cases of poisoning,12 cases of malignant tumor/hematologi⁃cal system disease,11 cases of circulatory failure,3 cases of biliary diseases,1 case of heat stroke disease,and 78 cases of unknown causes(33.0%).Among the 236 pediatric patients,131 were males(55.5%),and 109 cases were 0-1 year old(46.2%);there were 160 cases of jaundice(67.8%),148 cases of multiple organ dysfunction syndrome(MODS),118 cases of bleeding,80 cases of ascitic fluid,75 cases of hepatic encephalopathy,67 cases of disseminated intravascu⁃lar coagulation(DIC),and 20 cases of hepatorenal syndrome.In our study,87 cases(36.9%)survived,and 149 cases(63.1%)were invalid.Multivariate Logistic regression analysis showed that prolonged prothrombin time(PT)(OR=1.023,95%CI:1.003-1.044,P=0.025),increased blood ammonia(NH3)(OR=1.008,95%CI:1.002-1.014,P=0.011),decreased albumin(ALB)(OR=0.928,95%CI:0.872-0.989,P=0.030)and combined MODS(OR=2.980,95%CI:1.393-6.377,P=0.005)were risk factors for PALF.Conclusions This study found infectious diseases were the most common causes of PALF.PALF mainly occurred in male infants under 1 year old,with clinical features of jaun⁃dice,MODS,and bleeding,and they had poor prognosis.Children with prolonged PT,high NH3 level,and low ALB lev⁃el,and combined MODS had poorer outcomes.

关 键 词：肝衰竭 急性肝衰竭 多器官功能障碍综合征 感染性疾病 凝血酶原时间 白蛋白 

分 类 号：R575[医药卫生—消化系统]