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Fulminant hepatic failure: pediatric aspects
1Department of Child Health, King's College Hospital, London, United Kingdom.
Insights
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.
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.
Abstract:
In children, fulminant hepatic failure is a rare multisystem disorder in which severe impairment of liver function, with or without encephalopathy, occurs in association with hepatocellular necrosis in a patient with no recognized underlying chronic liver disease. Recognized etiologies include infections, toxins, metabolic disorders, infiltrative diseases, autoimmune hepatitis, ischemic or irradiation damage; a proportion of cases are cryptogenic. The diagnosis of the cause is essential to institute lifesaving medical treatment, decide if transplantation is indicated, and offer genetic counseling. The maximum International Normalized Ratio *(INR) reached during the course of the illness is the most sensitive predictor of outcome, mortality being 86% with an INR > or = 4, and 27% with an INR < 4 in our own series. Prognosis is worse in children younger than 2 years. Thus, urgent transplantation should be considered when the INR reaches 4, particularly in very young children. Survival after transplantation is 60% to 68%. Children with fulminant hepatic failure must be treated in specialized centers with facilities for liver transplantation.