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

Abstract

Objectives-To determine short-term outcome for children with acute liver failure (ALF) as it relates to etiology, clinical status, patient demographics and to determine prognostic factors.Study design-A prospective, multi-center 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 INR remained ≥ 20 seconds and/or >2, respectively, despite vitamin K. Primary outcome measures three weeks after study entry were death, death after transplant, alive with native liver, alive with transplanted organ.Results-The etiology of ALF in 348 children included acute acetaminophen toxicity (14%), metabolic disease (10%), autoimmune liver disease (6%), non-APAP 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 developed clinical encephalopathy.Conclusions-Etiologies of ALF in children differ from adults. Clinical encephalopathy may not be present in children. The high percentage of indeterminate cases provides an opportunity for investigation.Corresponding Address: Robert H. Squires, Jr., M.D., Professor of Pediatrics, University of Pittsburgh, Children's Hospital of Pittsburgh, 3705 Fifth Avenue, Pittsburgh, PA 15213, Phone: 412-692-8181, Fax: 412-692-7355, Email: Robert.squires@chp.edu. edited by WFB Publisher's Disclaimer: This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final citable form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain. (11), development of a reliable prognostic score will be useful in allocating organs to the most needy patients. NIH Public AccessThe Pediatric Acute Liver Failure (PALF) study group was formed in 1999 to develop a database that would facilitate an improved understanding of the etiopathogenesis, treatment and outcome of ALF in children. These data will also serve to identify factors that will help to predict the likelihood of death or need for liver transplant. Methods OrganizationThe PALF study group began as an adjunct to the Data CollectionFollowing informed consent from a parent or legal guardian, demographic, clinical and laboratory information were recorded daily for seven days. In most patients, an additional aliquot of serum or plasma was collected on each of the seven study days, frozen at −70° C and then shipped to the Data Coordinating Center (DCC) located at the University of Texas Southwestern Medical Center in Dallas, Texas. Diagnostic evaluation and medical management were consistent with the...

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