Abstract
Background
Identifying the causes of acute liver failure (ALF) and predictors of death or liver
transplantation (LTX) is crucial to decide its management. We aimed to describe features
and outcome of ALF in Italian children.
Methods
Retrospective review of cases presenting between 1996–2012. ALF was defined by high
transaminases, INR ≥2.0 regardless of hepatic encephalopathy (HE), no evidence of
underlying chronic liver disease.
Results
55 children (median age 2.6 years, range 0.1–15.1; M/F = 31/24) had ALF due to autoimmune hepatitis (AIH) in 10 (18%), metabolic disorders
in 9 (17%), paracetamol overdose in 6 (11%), mushroom poisoning in 3 (5%), viral infection
in 1 (2%), indeterminate in 26 (47%); 25/55 recovered with supportive management (45%);
28/55 underwent LTX and 2 died on the waiting list (55%). On multivariate analysis
severity of HE grade 3–4 and bilirubin ≥12 mg/dl were independent predictors of death or LTX (p < 0.05). After a median follow up of 4 years (range 2–15.0 years) the overall survival
rate was 93%.
Conclusion
Children with ALF can be managed successfully with combined medical treatment and
transplantation, warranting a survival rate similar to children transplanted because
of chronic conditions. In our cohort of patients severe HE and high bilirubin on admission
were independent predictors of the need of LTX.
Keywords
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References
- Acute liver failure in children: the first 348 patients in the pediatric acute liver failure study group.J Pediatr. 2006; 148: 652-658
- Acute liver failure in children.Clin Liver Dis. 2006; 10: 149-168
- Acute liver failure children.Clin Res Hepatol Gastroenterol. 2011; 35: 430-437
- Approaches to acute liver failure in children.Pediatr Transplant. 2004; 8: 584-588
- Etiology, outcome and prognostic indicators of childhood fulminant hepatic failure in the United Kingdom.J Pediatr Gastroenterol Nutr. 2005; 40: 575-581
- Nitisinone in the treatment of hereditary tyrosinaemia type 1.Drugs. 2006; 66: 743-750
- Pediatric liver transplantation for metabolic liver disease: experience at King’s College Hospital.Transplantation. 2009; 87: 87-93
- The pathophysiology and treatment of hereditary tyrosinemia type 1.Semin Liver Dis. 2001; 21: 563-571
- Acute liver failure in neonates, infants and children.Expert Rev Gastroenterol Hepatol. 2011; 5: 717-729
- Acute liver failure in infancy: a 14-year experience of a pediatric liver transplantation center.J Pediatr. 2001; 139: 871-876
- Acute liver failure in infancy [absctract].J Hepatol. 1995; 23: 125
- Acute liver failure in children and adolescents.Clin Res Hepatol Gastroenterol. 2012; 36: 278-283
- Acute liver failure in children: 20-year experience.Turk J Gastroenterol. 2012; 23: 127-134
- Autoimmune hepatitis.J Pediatr Gastroenterol Nutr. 2009; 49: 158-164
- Etiology and prognosis of acute liver failure in children.Liver Transpl. 2008; 14: S80-S84
- The continuing challenge of indeterminate acute liver failure.J Pediatr. 2009; 155: 769-770
- Wilson’s disease in children: 37-year experience and revised King’s score for liver transplantation.Liver Transpl. 2005; 11: 441-448
- Autoimmune hepatitis.Nat Rev Gastroenterol Hepatol. 2011; 8: 320-329
- Walker W.A. Durie P.R. Hamilton J.R. Pediatric gastrointestinal disease, pathophysiology, diagnosis, management. BC Decker, Ontario2000: 179-225
- Hepatic encephalopathy–definition, nomenclature, diagnosis, and quantification: final report of the working party at the 11th World Congresses of Gastroenterology, Vienna, 1998.Hepatology. 2002; 35: 716-721
- Hepatic encephalopathy: a review.Ann Hepatol. 2003; 2: 122-130
- Neonatal haemochromatosis with reversible pituitary involvement.Transpl Int. 2014; 27: 76-79
- King's College Hospital Criteria for non-acetaminophen induced acute liver failure in an international cohort of children.J Pediatr. 2013; 162: 319-323
- Pediatric acute liver failure: etiology, outcomes, and the role of serial pediatric end-stage liver disease scores.Pediatr Transplant. 2013; 17: 362-368
- Etiology and prognostic factors of acute liver failure in children.Indian Pediatr. 2013; 50: 677-679
- Fulminant hepatic failure: Wilson's disease or autoimmune hepatitis? Implications for transplantation.Pediatr Transplant. 2005; 9: 112-116
- Utility of simplified criteria for the diagnosis of autoimmune hepatitis in children.J Pediatr Gastroenterol Nutr. 2011; 52: 470-473
- Pattern of diagnostic evaluation for the causes of pediatric acute liver failure: an opportunity for quality improvement.J Pediatr. 2009; 155: 801-806
- The significance of autoantibodies and immunoglobulins in acute liver failure: a cohort study.J Hepatol. 2007; 47: 664-670
- Acute sporadic non-A, non-B, non-C, non-D, non-E hepatitis.Hepatology. 1997; 25: 478-483
- Outcomes following liver transplantation for seronegative acute liver failure: experience during a 12-year period with more than 100 patients.Liver Transpl. 2005; 11: 27-34
- Non-A, non-B fulminant hepatic failure.Arch Intern Med. 2001; 9: 1013-1014
- Association of fulminant non-A non-B hepatitis with homozygosity for HLA A1-B8-DR3.J Gastroenterol Hepatol. 2005; 20: 555-561
- Autoimmune acute liver failure: proposed clinical and histological criteria.Hepatology. 2011; 53: 517-526
- Steroid use in acute liver failure.Hepatology. 2014; 59: 612-621
- Optimizing management in autoimmune hepatitis with liver failure at initial presentation.World J Gastroenterol. 2011; 17: 2070-2075
- Efforts at making the diagnosis of acute-onset autoimmune hepatitis.Hepatology. 2011; 54: 371-372
- Autoimmune acute liver failure.Hepatology. 2011; 54: 372-373
- Acute liver failure in children.Semin Liver Dis. 2008; 28: 153-166
- Delayed encephalopathy in fulminant hepatic failure in the pediatric population and the role of liver transplantation.J Pediatr Gastroenterol Nutr. 1997; 24: 128-134
- Fulminant hepatic failure in childhood: an analysis of 31 cases.Arch Dis Child. 1980; 55: 252-258
- Successful management of neonatal hemochromatosis by exchange transfusion and immunoglobulin: a case report.J Perinatol. 2013; 33: 83-85
Article info
Publication history
Published online: June 14, 2017
Accepted:
May 30,
2017
Received in revised form:
April 18,
2017
Received:
January 27,
2017
Identification
Copyright
© 2017 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevier Ltd. All rights reserved.
