Premium
Factors that predict mortality in children with W ilson disease associated acute liver failure and comparison of W ilson disease specific prognostic indices
Author(s) -
Devarbhavi Harshad,
Singh Rajvir,
Adarsh Channagiri K,
Sheth Keyur,
Kiran Ravi,
Patil Mallikarjun
Publication year - 2014
Publication title -
journal of gastroenterology and hepatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.214
H-Index - 130
eISSN - 1440-1746
pISSN - 0815-9319
DOI - 10.1111/jgh.12356
Subject(s) - medicine , hazard ratio , proportional hazards model , liver disease , model for end stage liver disease , gastroenterology , hepatic encephalopathy , univariate analysis , stepwise regression , creatinine , encephalopathy , cirrhosis , confidence interval , multivariate analysis , liver transplantation , transplantation
Background and Aims W ilson 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 C ox regression analysis were analyzed by forward stepwise C ox hazards regression. The prognostic models such as N azer's model, revised K ings C ollege M odel, 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 C ox 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 N azer index, revised K ing's C ollege C riteria, 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, N azer index and K ings C ollege C riteria are comparable with a AUC between 0.74–0.76.