Pediatric Wilson Disease Presenting as Acute Liver Failure: An Individual Patient Data Meta-analysis.

Journal of Pediatric Gastroenterology and Nutrition
Shannon M VandrielBinita M Kamath

Abstract

Wilson disease (WD) presenting as acute liver failure (ALF) is rare and typically fatal without liver transplantation (LT). Its rarity has hindered comprehensive studies. We undertook an individual patient data meta-analysis to characterize a cohort of pediatric patients presenting with ALF whose final diagnosis was WD to examine outcomes and identify predictors of poor outcomes. Database searches were conducted in PubMed, ScienceDirect, and Google Scholar, restricted to English-language articles published between January 1984 and May 2018. Articles were excluded if pediatric (<18 years old) data were not extractable or if LT was not readily available at reporting institutions. Extracted data included clinical and biochemical characteristics, genotype, treatment, and outcome. Data were available on 249 subjects from 52 articles, plus 7 additional subjects identified from our institution's WD database (N = 256). Females represented 69% (n = 170/245). Median age at presentation was 13.4 years (n = 204, range 4.0-17.9). Of the total 256 subjects, 87% underwent LT, 11% achieved spontaneous recovery and 2% died before LT. International normalized ratio >2.0 at presentation was a predictor of LT/death (odds ratio 7.6, 95% confidence ...Continue Reading

References

Apr 1, 1979·American Journal of Diseases of Children·E J DoeringT E Dittmer
Aug 1, 1977·American Journal of Diseases of Children·R AdlerR G Robinson
Nov 1, 1992·Hepatology : Official Journal of the American Association for the Study of Liver Diseases·R SallieR Williams
Feb 1, 1996·The Journal of Pediatrics·E E Santos SilvaE M Sokal
Nov 4, 2000·The Journal of Pediatrics·D C WilsonE A Roberts
Dec 18, 2002·Annals of Internal Medicine·George OstapowiczUNKNOWN U.S. Acute Liver Failure Study Group
Jul 2, 2003·Pediatric Critical Care Medicine : a Journal of the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies·Pierre TissièresDenis Devictor
Sep 6, 2003·Liver International : Official Journal of the International Association for the Study of the Liver·Peter FerenciFrieder Berr
Apr 28, 2007·World Journal of Gastroenterology : WJG·Christoph EisenbachUta Merle
May 29, 2008·Hepatology : Official Journal of the American Association for the Study of Liver Diseases·Eve A RobertsUNKNOWN American Association for Study of Liver Diseases (AASLD)
Sep 18, 2008·Hepatology : Official Journal of the American Association for the Study of Liver Diseases·Jessica D KormanUNKNOWN Pediatric and Adult Acute Liver Failure Study Groups
May 24, 2012·Metallomics : Integrated Biometal Science·Eve A Roberts
Feb 13, 2014·Annals of the New York Academy of Sciences·Peter Ferenci
Apr 29, 2015·JAMA : the Journal of the American Medical Association·Lesley A StewartUNKNOWN PRISMA-IPD Development Group
Jun 5, 2015·World Journal of Pediatrics : WJP·Yi TianFeng Peng
Aug 15, 2015·Journal of Clinical Apheresis·Huy P PhamYanyun Wu
Jul 2, 2016·Journal of Pediatric Gastroenterology and Nutrition·Vicky L NgUNKNOWN Pediatric Acute Liver Failure Study Group (PALFSG)
Oct 16, 2016·European Journal of Neurology : the Official Journal of the European Federation of Neurological Societies·A PoujoisF Woimant
Feb 17, 2017·BMC Medical Research Methodology·Doneal ThomasAndrea Benedetti
Mar 19, 2019·Transplantation Proceedings·Z GaroufaliaG C Sotiropoulos
Dec 16, 2019·Liver Transplantation : Official Publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society·Michelle A CamarataMichael L Schilsky

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Citations

Mar 30, 2021·Digestive and Liver Disease : Official Journal of the Italian Society of Gastroenterology and the Italian Association for the Study of the Liver·A Di GiorgioL D'Antiga
Jun 7, 2021·Journal of Pediatric Gastroenterology and Nutrition·Eduardo CouchonnalAlain Lachaux

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