Characterization and outcomes of young infants with acute liver failure

Shikha S Sundaram, Estella M Alonso, Michael R Narkewicz, Song Zhang, Robert H Squires, Pediatric Acute Liver Failure Study Group, Saul Karpen, Ruben E Quiros, Dominic Dell Olio, Deirdre Kelly, Robert Squires, Ben Schneider, Steven Lobritto, Humberto Soriano, Rene Romero, Scott Elisofon, Maureen Jonas, Vick Ng, Girish Subbarao, Kathleen Schwartz, Anil Dhawan, Nanda Kerkar, Sukru Emre, Estella Alonso, Brendan M McGuire, Martin G Martin, Joel E Lavine, Philip Rosenthal, John Bucuvalas, Nada Yazigi, Michael Narkewicz, M James Lopez, Simon Horslen, Liz Rand, Daniel W Thomas, Norberto Rodriguez-Baez, Karen Murray, David Rudnick, Ross Shepherd, Shikha S Sundaram, Estella M Alonso, Michael R Narkewicz, Song Zhang, Robert H Squires, Pediatric Acute Liver Failure Study Group, Saul Karpen, Ruben E Quiros, Dominic Dell Olio, Deirdre Kelly, Robert Squires, Ben Schneider, Steven Lobritto, Humberto Soriano, Rene Romero, Scott Elisofon, Maureen Jonas, Vick Ng, Girish Subbarao, Kathleen Schwartz, Anil Dhawan, Nanda Kerkar, Sukru Emre, Estella Alonso, Brendan M McGuire, Martin G Martin, Joel E Lavine, Philip Rosenthal, John Bucuvalas, Nada Yazigi, Michael Narkewicz, M James Lopez, Simon Horslen, Liz Rand, Daniel W Thomas, Norberto Rodriguez-Baez, Karen Murray, David Rudnick, Ross Shepherd

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.

Conflict of interest statement

The authors declare no conflicts of interest.

Copyright © 2011 Mosby, Inc. All rights reserved.

Figures

Figure 1
Figure 1
The cumulative incidence of transplantation by etiology in infants 0-90 days of age in the PALF Study.
Figure 2
Figure 2
The cumulative incidence of death without transplantation by etiology in infants 0-90 days of age in the PALF Study.

Source: PubMed

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