Pediatric Wilson disease presenting as acute liver failure: Prognostic indices

作者：Weiyuan Fang, Kuerbanjiang Abuduxikuer, Peng Shi, Yi‐Ling Qiu, Jing Zhao, Yu-Chuan Li, Xueyuan Zhang, Neng‐Li Wang, Xin‐Bao Xie, Yi Lu, A. S. Knisely, Jian‐She Wang · 发表于：World Journal of Clinical Cases · 年份：2021 · DOI：10.12998/wjcc.v9.i14.3273 · 被引用次数：17 · 研究领域：Trace Elements in Health、Liver Disease and Transplantation、Metabolism and Genetic Disorders

BACKGROUND: Acute liver failure (ALF) can be a primary presentation of Wilson disease (WD). Mortality rates are high in WD with ALF (WDALF). Predictions of mortality in WDALF vary by model and are sometimes contradictory, perhaps because few patients are studied or WD diagnoses are questionable. AIM: To determine the outcomes among well-documented WDALF patients and assess mortality model performance in this cohort. METHODS: = 41 over 6-years-old, single-center retrospective study) and compared seven prognostic models (King's College Hospital Criteria, model for end-stage liver disease/pediatric end-stage liver disease scoring systems, Liver Injury Unit [LIU] using prothrombin time [PT] or international normalized ratio [INR], admission LIU using PT or INR, and Devarbhavi model) with one another. RESULTS: variants in 36. In 5 others, Kayser-Fleischer rings and Coombs-negative hemolytic anemia permitted diagnosis. Three died during hospitalization and three underwent liver transplantation (LT) within 1 mo of presentation and survived (7.3% each); 35 (85.4%) survived without LT when given enteral D-penicillamine and zinc-salt therapy with or without urgent plasmapheresis. Parameters significantly correlated with mortality included encephalopathy, coagulopathy, and gamma-glutamyl transpeptidase activity, bilirubin, ammonia, and serum sodium levels. Area under the receiver operating curves varied among seven prognostic models from 0.981 to 0.748 with positive predictive values fr...