Characterization and outcomes of young infants with acute liver failure

J Pediatr. 2011 Nov;159(5):813-818.e1. doi: 10.1016/j.jpeds.2011.04.016. Epub 2011 May 31.

Abstract

Objective: To characterize infants aged ≤ 90 days enrolled in an international, multicenter, prospective registry of children aged < 18 years with acute liver failure (ALF).

Study design: The Pediatric Acute Liver Failure (PALF) Study Group collects prospective data on children from birth to 18 years. We analyzed data from infants aged ≤ 90 days enrolled in the PALF Study before May 18, 2009.

Results: A total of 148 infants were identified in the PALF registry (median age, 18 days). Common etiologies of ALF were indeterminate (38%), neonatal hemochromatosis (13.6%), and herpes simplex virus (12.8%). Spontaneous survival occurred in 60% of the infants, 16% underwent liver transplantation, and 24% died without undergoing liver trsansplantation. Infants with indeterminate ALF were more likely to undergo liver transplantation than those with viral-induced ALF (P = .0002). The cumulative incidence of death without liver transplantation was higher in infants with viral ALF (64%) compared with those with neonatal hemochromatosis (16%) or indeterminate ALF (14%) (P = .0007).

Conclusion: ALF in young infants presents unique diagnostic considerations. Spontaneous survival is better than previously thought. Liver transplantation provides an additional option for care.

Publication types

  • Multicenter Study
  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Alanine Transaminase / blood
  • Aspartate Aminotransferases / blood
  • Cholestasis / epidemiology
  • Hemochromatosis / congenital
  • Hemochromatosis / epidemiology
  • Hepatic Encephalopathy / epidemiology
  • Herpes Simplex / epidemiology
  • Humans
  • Hyperbilirubinemia / epidemiology
  • Infant
  • Infant, Newborn
  • International Normalized Ratio
  • Liver Failure, Acute / etiology
  • Liver Failure, Acute / mortality*
  • Liver Failure, Acute / surgery*
  • Liver Transplantation / statistics & numerical data*
  • Prospective Studies
  • Registries
  • gamma-Glutamyltransferase / blood

Substances

  • gamma-Glutamyltransferase
  • Aspartate Aminotransferases
  • Alanine Transaminase