Wilson病相关慢加急性肝衰竭患者预后影响因素及其预测价值  

Prognostic factor and its predictive value of patients with Wilson's disease-related acute-on-chronic liver failure

作　　者：唐露露 陈怀珍[1] 张静[1] 董婷[1] 李俊[1] 江海林 杨文明[1] Tang Lu-Lu;Chen Huai-Zhen;Zhang Jing;Dong Ting;Li Jun;Jiang Hai-Lin;Yang Wen-Ming(Department of Encephalopathy,the First Affiliated Hospital of Anhui University of Chinese Medicine,Hefei,Anhui 230031,China)

机构地区：[1]安徽中医药大学第一附属医院脑病科,安徽合肥230031

出　　处：《解放军医学杂志》2024年第2期131-136,共6页Medical Journal of Chinese People's Liberation Army

基　　金：国家自然科学基金(81973825);安徽省自然科学基金(2208085MH270,2108085QH366);安徽省教育厅重点项目(KJ2021A0547)。

摘　　要：目的探讨Wilson病(WD)相关慢加急性肝衰竭(ACLF)患者的预后影响因素及其预测价值。方法收集2017年1月1日-2022年1月1日在安徽中医药大学第一附属医院脑病科住院的70例WD-ACLF患者的临床资料进行回顾性分析,根据12周预后情况分为生存组(n=36)与死亡组(n=34)。采用单因素和多因素logistic回归分析WD-ACLF患者预后的影响因素,建立预后模型,对模型整体系数进行综合检验,采用Hosmer-Lemeshow检验评价模型的拟合度。采用ROC曲线分析新建模型以及慢性肝衰竭-序贯器官衰竭评估(CLIF-SOFA)评分、终末期肝病模型(MELD)评分、肝硬化分级(CTP)评分对WD-ACLF预后的预测价值。结果共纳入70例WD-ACLF患者,生存组36例,男22例,女14例,中位年龄30.0(17.3,40.0)岁;死亡组34例,男25例,女9例,中位年龄34.0(28.8,41.0)岁。单因素分析结果显示,生存组病程、凝血酶原时间(PT)、活化部分凝血活酶时间(APTT)短于死亡组,白细胞计数(WBC)、国际化标准比值(INR)、天冬氨酸转氨酶(AST)、总胆红素(TBIL)、尿素氮(BUN)、肌酐(Cre)、铜蓝蛋白(CER)水平,以及感染、腹水和上消化道出血比例低于死亡组,红细胞计数(RBC)及血红蛋白(Hb)、Na^(+)、总胆固醇(TC)水平高于死亡组(P<0.05或P<0.01)。多因素logistic回归分析结果显示,病程(OR=1.176,95%CI1.043~1.325)、INR(OR=7.635,95%CI1.767~32.980)、TBIL(OR=1.012,95%CI1.003~1.021)、上消化道出血(OR=11.654,95%CI 1.029~131.980)是影响WD-ACLF预后的独立危险因素(P<0.05)。根据logistic回归分析结果建立WD-ACLF预后联合模型,该模型评估WD-ACLF预后的AUC为0.941,大于CLIF-SOFA评分(AUC=0.802)、MELD评分(AUC=0.897)、CTP评分(AUC=0.722)。结论病程、INR、TBIL、上消化道出血是影响WDACLF预后的危险因素,据此建立的预后模型能更为准确地预测WD-ACLF患者的预后,其预测价值优于CLIF-SOFA评分、MELD评分和CTP评分。Objective To explore the prognostic factor and its predictive value of patients with Wilson disease-related acuteon-chronic liver failure(WD-ACLF).Methods The clinical data of 70 patients diagnosed as WD-ACLF admitted to the Department of Encephalopathy of the First Affiliated Hospital of Anhui University of Chinese Medicine from January 1,2017 to January 1,2022 were retrospectively collected.According to the 12-week prognosis,patients were divided into survival group(n=36)and death group(n=34).The data of the two groups were analyzed by univariate and multivariate logistic analysis to screen the prognostic risk factors and evaluate their predictive value.The model coefficient is omnibus tested,and the model-fitting degree is evaluated by the Hosmer-Lemeshow test.ROC curve was used to analyze the prognostic value for WD-ACLF between the new model and chronic liver failure-sequential organ failure assessment(CLIF-SOFA)score,model for end-stage liver disease(MELD)score and Child-Turcotte-Pugh(CTP)score.Results A total of 70 WD-ACLF patients were enrolled in present study,including 36 cases in survival group[22 males and 14 females with median age of 30.0(17.3,40.0)]and 34 cases in death group[25 males and 9 females with median age of 34.0(28.8,41.0)].Univariate analysis showed that the course of disease,prothrombin time(PT),activated partial thromboplastin time(APTT)were shorter in survival group than that in death group,the white blood cells(WBC),international normalized ratio(INR),aspartate transaminase(AST),total bilirubin(TBIL),blood urea nitrogen(BUN),creatinine(Cre)and ceruloplasmin(CER)levels and the proportion of infection,ascites,and upper gastrointestinal bleeding were lower in survival group than those in death group,however,the proportion of infection,ascites and upper digestive bleeding in the survival group were lower than those in the death group.Meanwhile,the red blood cells(RBC),hemoglobin(Hb),Na^(+)and total cholesterol(TC)level in the survival group were higher than those in the death group(P<0.0

关 键 词：WILSON病 慢加急性肝衰竭 预后 肝衰竭-序贯器官衰竭评估 终末期肝病模型 肝硬化分级 

分 类 号：R742.4[医药卫生—神经病学与精神病学]