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Fulminant hepatic failure: pediatric aspects

B R Bhaduri1, G Mieli-Vergani

  • 1Department of Child Health, King's College Hospital, London, United Kingdom.

Seminars in Liver Disease
|November 1, 1996
PubMed
Summary

Fulminant hepatic failure in children is a severe liver condition with various causes. An International Normalized Ratio (INR) of 4 or higher indicates a poor prognosis, suggesting urgent liver transplantation.

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Area of Science:

  • Pediatric Hepatology
  • Critical Care Medicine
  • Transplantation Surgery

Background:

  • Fulminant hepatic failure (FHF) in children is a rare, life-threatening condition characterized by severe liver dysfunction and hepatocellular necrosis without pre-existing chronic liver disease.
  • Etiologies are diverse, including infections, toxins, metabolic disorders, autoimmune conditions, and ischemic damage, with some cases remaining cryptogenic.
  • Accurate diagnosis is crucial for guiding treatment, determining the need for liver transplantation, and providing genetic counseling.

Purpose of the Study:

  • To highlight the critical role of the International Normalized Ratio (INR) as a prognostic marker in pediatric fulminant hepatic failure.
  • To emphasize the importance of timely liver transplantation in managing severe cases of FHF in children.
  • To underscore the necessity of specialized care for children with FHF.

Main Methods:

  • Retrospective analysis of pediatric patients diagnosed with fulminant hepatic failure.
  • Evaluation of the International Normalized Ratio (INR) as a predictor of mortality and need for transplantation.
  • Assessment of outcomes in patients undergoing liver transplantation versus those managed non-surgically.

Main Results:

  • The maximum INR achieved during illness is a sensitive predictor of outcome, with mortality rates of 86% for INR ≥ 4 and 27% for INR < 4.
  • Prognosis is significantly worse in children younger than 2 years.
  • Survival rates after liver transplantation range from 60% to 68%.

Conclusions:

  • Urgent liver transplantation should be strongly considered when INR reaches 4, especially in young children, due to high mortality.
  • Children with fulminant hepatic failure require management in specialized centers equipped for liver transplantation.
  • Early identification of prognostic indicators like INR is vital for optimizing patient outcomes in pediatric FHF.

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