肝豆状核变性肝硬化失代偿期患者预后及影响因素分析

Prognostic factors of hepatolenticular degeneration patients with decompensated liver cirrhosis

目的探讨肝豆状核变性(WD)肝硬化失代偿期患者的预后及影响预后的相关因素。方法回顾性分析2012年10月至2015年12月住院的67例WD合并肝硬化失代偿期患者的临床资料,根据生存结局分为存活组和死亡组,比较两组一般资料、生化指标、并发症、Child-Turcotte-Pugh(CTP)分值、终末期肝病模型(MELD)分值。结果死亡组患者16例,存活组患者51例,死亡组患者白蛋白(ALB)、胆碱酯酶(CHE)、血清钠均较存活组患者低,BMI、TBil、PT、国际标准比值(INR)、血氨、透明质酸(HA)、铜蓝蛋白(CP)、CTP分值、MELD分值均较存活组患者高,差异均有统计学意义(均P<0.05);死亡组患者更容易出现中等量以上的腹水、肝性脑病(HE)(均P<0.05);CTP分级(A级、B级、C级)及MELD分值(<5分和>5分)对于3年内患者的预后预测具有统计学差异(均P<0.01);经Cox比例风险模型分析,TBil、CHE、肝性脑病是WD肝硬化失代偿期患者预后的相对独立危险因素(P<0.05)。结论 WD肝硬化失代偿期患者出现高水平TBil、低水平CHE及肝性脑病,提示预后较差,CTP系统及MELD模型均能用于评估患者的预后。

Objective To investigate the prognostic factors of hepatolenticular degeneration(Wilson disease,WD)patients with decompensated liver cirrhosis.Methods Clinical data of 67 WD patients with decompensated liver cirrhosis admitted in hospital from October 2012 to December 2015 were retrospectively reviewed.The general information,biochemical index,complications,Child-Turcotte-Pugh(CTP)score and End-stage Liver Disease Model(MELD)score were compared between the fatal patients and survival patients,and the factors related to prognosis were analyzed.Results There were 16 patients in fatal group and 51 patients in the survival group.Albumin(ALB),cholinesterase(CHE)and serum sodium in the fatal group were lower than those in the survival group.Body mass index(BMI),total bilirubin(TBil),prothrombin time(PT),international standard ratio(INR),blood ammonia,hyaluronic acid(HA),ceruloplasmin(CP),CTP score and MELD score in the fatal group were higher than those in the survival group(all P<0.05).The ascites and hepatic encephalopathy(HE)were more likely to occur in the fatal group(P<0.05).The CTP classification(A,B,C)and MELD score(<5 and>5)were significantly associated with the prognosis of patients within three years(all P<0.01).The COX proportional risk model analysis showed that TBil,CHE and HE were independent risk factors for the prognosis of WD decompensated cirrhosis(P<0.05).Conclusion The high TBil level,low CHE level and the presence of hepatic encephalopathy indicate poor prognosis in WD patients with decompensated cirrhosis;the CTP score and MELD model can be also used to evaluate the prognosis of patients.