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Acute liver failure in children: the first 348 patients in the pediatric acute liver failure study group
Robert H Squires1, Benjamin L Shneider, John Bucuvalas
1University of Pittsburgh, Children's Hospital of Pittsburgh, PA 15213, USA. Robert.squires@chp.edu
Insights
Causes of pediatric acute liver failure (ALF) vary, with nearly half indeterminate. Outcomes differ by cause, and clinical encephalopathy may be absent in children with ALF, highlighting the need for further research.
Area of Science:
- Pediatric Hepatology
- Gastroenterology
- Critical Care Medicine
Background:
- Acute liver failure (ALF) in children presents unique challenges in diagnosis and management.
- Understanding the specific causes and prognostic factors in pediatric ALF is crucial for improving outcomes.
Purpose of the Study:
- To investigate the short-term outcomes of pediatric acute liver failure.
- To identify the causes, clinical status, and demographic factors associated with ALF in children.
- To determine prognostic factors for children diagnosed with ALF.
Main Methods:
- A prospective, multicenter case study was conducted.
- Demographic, clinical, laboratory, and short-term outcome data were collected for 348 children (birth to 18 years) with ALF.
- Primary outcomes included death, death after transplantation, survival with native liver, and survival with a transplanted organ within 3 weeks of study entry.
Main Results:
- The identified causes of ALF included acetaminophen toxicity (14%), metabolic disease (10%), autoimmune liver disease (6%), drug-related hepatotoxicity (5%), and infections (6%), with 49% of cases remaining indeterminate.
- Outcomes varied significantly across different patient subgroups.
- Notably, 20% of children with non-acetaminophen ALF experienced death or liver transplantation without prior clinical encephalopathy.
Conclusions:
- The etiology of acute liver failure in children differs from that observed in adult populations.
- Clinical encephalopathy may not be a universal indicator in pediatric ALF cases.
- The substantial proportion of indeterminate cases underscores the need for further etiological investigations in pediatric ALF.
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 > or = 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.
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