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Liver Failure in Early Infancy: Aetiology, Presentation, and Outcome

Rana Bitar1, Rosemary Thwaites, Suzanne Davison

  • 1*Paediatric Gastroenterology, Great North Children's Hospital, Newcastle †Paediatrics, James Cook University Hospital, Middlesbrough ‡Children's Liver Unit, Leeds Children's Hospital, Leeds, UK.

Journal of Pediatric Gastroenterology and Nutrition
|March 24, 2016
PubMed

Insights

Acute liver failure in infants is rare but recognizing it early is key. Even with normal liver enzymes, coagulopathy in young infants warrants investigation for potential liver disease.

Area of Science:

  • Pediatric Gastroenterology
  • Hepatology
  • Neonatal Medicine

Background:

  • Acute liver failure (ALF) in early infancy presents unique diagnostic and management challenges.
  • Early recognition is crucial for improving outcomes in affected infants.

Purpose of the Study:

  • To describe the clinical presentation and outcomes of infants with ALF.
  • To correlate presentation and outcomes with the underlying etiology of ALF.
  • To identify features aiding early recognition and prompt management.

Main Methods:

  • Retrospective review of infants presenting with liver failure within 120 days of birth over a 19-year period.
  • Collection of data on etiology, clinical features, investigations, and patient outcomes.
  • Analysis of presentation characteristics across different etiological groups.

Main Results:

  • 78 infants with ALF were identified; etiology was determined in 94%.
  • Metabolic disease (36%), hypoxic-ischemic insult (19%), and infection (17%) were common causes.
  • Jaundice was absent in 24% of infants; coagulopathy with near-normal transaminases/bilirubin occurred in some etiologies.
  • Hospital discharge survival was 58%, with 1-year survival at 53% (native liver survival 50%).
  • Liver transplantation was performed in 6 infants.

Conclusions:

  • ALF should be suspected in any young infant with coagulopathy, even with normal liver enzymes.
  • Improved intensive care and liver transplantation have potentially improved outcomes compared to earlier periods.
  • Prompt diagnosis and management based on etiology are vital for better infant survival rates.
Abstract

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