Elsevier

The Journal of Pediatrics

Volume 159, Issue 5, November 2011, Pages 813-818.e1
The Journal of Pediatrics

Original Article
Characterization and Outcomes of Young Infants with Acute Liver Failure

https://doi.org/10.1016/j.jpeds.2011.04.016Get rights and content

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.

Section snippets

Methods

Data were obtained from the PALF Study Group, a multicenter, prospective study initiated in 1999 that collects data on children aged <18 years with ALF from 24 participating centers in the United States, Canada, and the United Kingdom. Entry criteria include evidence of acute liver injury combined with either severe coagulopathy (International Normalized Ratio [INR] >2.0 or prothrombin time [PT] >20 seconds) or encephalopathy with moderate coagulopathy (INR ≥1.5 or PT ≥15 seconds).

Results

Of the 841 subjects enrolled in the PALF registry, 148 were aged ≤90 days at enrollment (57.4% males; 73.0% Caucasian). The median age at enrollment was 18 days (Q1, 11 days; Q3, 37 days). The median weight at enrollment was 3.5 kg (Q1, 2.8 kg; Q3, 4.2 kg), and the median weight-for-age z score was –1.3 (Q1, –2; Q3, –0.2). The smallest infant weighed 1.7 kg (weight-for-age z score, –3.10), and the largest infant weighed 6.4 kg (weight-for-age z score, 3.32). The most common presenting symptoms

Discussion

This large prospective multicenter study characterized ALF in young infants. As might be expected, the etiology of ALF differed between young infants and older pediatric patients. The most common cause of ALF in young infants was NH, a disease limited to the neonatal period. Viral infections, mainly herpes simplex, were prominent as well (16%). A variety of metabolic diseases, dominated by galactosemia, also caused ALF. In this series, respiratory chain defects were more common than

References (20)

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PALF Study Group member institutions are supported by the National Institutes of Health (Grants U01-DK 072146-04, ULI RR025014-01, UL1 RR024131, and UL1 RR024153) and General Clinical Research Centers (Grants M01 RR 08084, to the University of Cincinnati, and MO1 RR00069, to the University of Colorado). The authors declare no conflicts of interest.

List of members of the Pediatric Acute Liver Failure Study Group is available at www.jpeds.com (Appendix).

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