Factors that predict mortality in children with Wilson disease associated acute liver failure and comparison of Wilson disease specific prognostic indices
Factors that predict mortality in children with Wilson disease associated acute liver failure and comparison of Wilson disease specific prognostic indices
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DOI：
10.1111/jgh.12356
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发表时间：
2014-02-01
影响因子：
4.1
通讯作者：
Patil, Mallikarjun
中科院分区：
文献类型：
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作者：
Devarbhavi, Harshad;Singh, Rajvir;Patil, Mallikarjun
背景和目的：肝豆状核变性(WD)相关的急性肝功能衰竭(ALF)对儿童的影响大于成人。没有脑病的患者的死亡率和预后的预测因素尚不清楚。我们探讨了终末期肝病模型(MELD)等多种模型预测WD和ALF患儿死亡率的能力。方法：分析61例18岁WD和ALF患儿的住院特征。单因素COX回归分析中与死亡率相关的因素采用前向逐步COX危险回归分析。结果：145例18岁WD患儿中，61例发生ALF，死亡33例(54%)，其中脑病22例(81.5%)，非脑病11例(32.4%)。ALF患儿平均年龄为9.7岁，其中38例(62.3%)为男孩。对死亡率有显著影响的预后因素包括脑病、国际标准化比率、总蛋白、总胆红素和直接胆红素、碱性磷酸酶、血清肌酐和白细胞计数。前瞻性逐步COX比例风险回归确定脑病(危险比2.88；CI 1.1-7.4)和总胆红素(危险比1.05；CI：1.02-1.09)是预后的预测因素。Nazer指数、修订的King‘s College标准和PELD/MELD的受试者工作曲线下面积(AUC)分别为0.74、0.76和0.75。结论：WD和ALF的病死率为54%，其中脑病为81.5%，非脑病为32.4%。预后模型、MELD/PELD评分、Nazer指数和Kings College标准与AUC在0.74-0.76之间具有可比性。
Background and Aims: Wilson disease (WD) associated acute liver failure (ALF) affects children more than adults. The predictors of mortality and outcome in patients without encephalopathy are not clear. We investigated the ability of prognostic factors and various models including model for end-stage liver disease (MELD) to predict mortality among children with WD and ALF.Methods: We analyzed the admission characteristics in 61 children < 18 years with WD and ALF. Factors associated with mortality on univariate Cox regression analysis were analyzed by forward stepwise Cox hazards regression. The prognostic models such as Nazer's model, revised Kings College Model, and pediatric end-stage liver disease/model for end-stage liver disease (PELD/MELD) score were compared.Results: Of the 145 children < 18 years with WD, 61 experienced ALF of whom 33 (54%) died, including 22/27 (81.5%) with encephalopathy and 11/34 (32.4%) without encephalopathy. The mean age of children with ALF was 9.7 years, 38(62.3%) were boys. Prognostic factors significant for mortality included encephalopathy, international normalized ratio, total proteins, total and direct bilirubin, alkaline phosphatase, serum creatinine, and white blood cell count. Forward stepwise Cox proportional hazards regression identified encephalopathy (hazard ratio 2.88; CI 1.1-7.4) and total bilirubin (hazard ratio 1.05; CI: 1.02-1.09) as predictors of outcome. The area under the receiver operating curve (AUC) of the Nazer index, revised King's College Criteria, and PELD/MELD were 0.74, 0.76, and 0.75, respectively.Conclusions: Mortality in children with WD and ALF is 54% including 81.5% with encephalopathy and 32.4% without encephalopathy. The prognostic models, MELD/PELD score, Nazer index and Kings College Criteria are comparable with a AUC between 0.74-0.76.