Original article
Acute liver failure in children: The first 348 patients in the pediatric acute liver failure study group

https://doi.org/10.1016/j.jpeds.2005.12.051Get rights and content

Objectives

To determine short-term outcome for children with acute liver failure (ALF) as it relates to cause, clinical status, and patient demographics and to determine prognostic factors.

Study design

A prospective, multicenter case study collecting demographic, clinical, laboratory, and short-term outcome data on children from birth to 18 years with ALF. Patients without encephalopathy were included if the prothrombin time and international normalized ratio remained ≥20 seconds and/or >2, respectively, despite vitamin K. Primary outcome measures 3 weeks after study entry were death, death after transplantation, alive with native liver, and alive with transplanted organ.

Results

The cause of ALF in 348 children included acute acetaminophen toxicity (14%), metabolic disease (10%), autoimmune liver disease (6%), non-acetaminophen drug-related hepatotoxicity (5%), infections (6%), other diagnosed conditions (10%); 49% were indeterminate. Outcome varied between patient sub-groups; 20% with non-acetaminophen ALF died or underwent liver transplantation and never had clinical encephalopathy.

Conclusions

Causes of ALF in children differ from in adults. Clinical encephalopathy may not be present in children. The high percentage of indeterminate cases provides an opportunity for investigation.

Section snippets

Organization

The PALF study group began as an adjunct to the National Institutes of Health–sponsored, adult-focused ALF Study Group (William Lee, MD, Principal Investigator). The PALF study group now consists of 24 active pediatric sites, 21 within the United States, 1 in Canada, and 2 in the United Kingdom. Working groups of pediatric hepatologists established definitions for ALF and various diagnostic categories. Representatives from all participating centers approved final recommendations from the

Results

Between December 1999 and December 2004, 348 children were enrolled; patient demographics are outlined in Table II. The median dose of acetaminophen ingested was 183 mg/kg (range 19.2 to 734.1). There was an association between cause and sex (P = .0002), with the percentage of females significantly higher in the acetaminophen group compared with both non-acetaminophen groups (TTMC P < .05, 79% vs 46% or vs 60%, respectively). The association between the three etiologic categories and race

Discussion

This report of the first 348 children in the PALF data set highlights a number of important observations: (1) HE is not an absolute requirement to establish the diagnosis of ALF in children; (2) a specific diagnosis was not made in almost half of all infants and children; (3) the causes of ALF in children differ from those seen in adults,16 with children having more indeterminate cases and fewer acetaminophen and viral-induced cases; and (4) short-term outcome varied among diagnostic groups.

HE

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    Supported by NIH grant RO1-DK58369-01; and by M01-RR00069, M01-RR00037, and M01 RR08084 from the General Clinical Research Center Program of the National Center for Research Resources of the NIH.

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