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Published on: July 1, 2012
Bridging to transplantation in acute liver failure in a 7-month-old infant
Gerhard Trittenwein1, Harald Boigner, Gehan Mostafa
1PICU, University of Vienna, and Department of Clinical Pathology, University Children's Hospital, University of Vienna, Austria. gerhard.trittenwein@meduniwien.ac.at
Insights
Molecular adsorbent recycling system (MARS) albumin dialysis successfully bridged a 7-month-old infant with acute liver failure to a split-liver transplant. This artificial detoxification improved encephalopathy, amino acid levels, and hemodynamics, leading to a good long-term outcome.
Area of Science:
- Pediatric Hepatology
- Critical Care Medicine
- Transplantation Surgery
Background:
- Acute liver failure (ALF) in children is a rare, life-threatening condition where liver transplantation is often the only definitive treatment.
- Bridging therapy with artificial detoxification methods is crucial for patients with deteriorating hepatic encephalopathy awaiting liver transplantation.
- Albumin dialysis using the molecular adsorbent recycling system (MARS) has shown efficacy in adults as a bridge to liver transplantation.
Observation:
- A 7-month-old infant with ALF due to autoimmune hepatitis met King's College criteria for transplantation.
- The infant experienced hemodynamic and neurological deterioration, including grade III encephalopathy, despite moderate hyperammonemia and pneumonia.
- MARS albumin dialysis was initiated as a 36-hour bridge to living-donor split-liver transplantation.
Findings:
- Albumin dialysis with MARS led to significant improvements in the infant's electroencephalogram (EEG), plasma amino acid levels, and hemodynamic status.
- The infant underwent successful living-donor split-liver transplantation.
- Twenty-four months post-transplant, the child exhibits normal liver function and neuropsychological development.
Implications:
- Albumin dialysis using MARS is a viable and successful bridging therapy for infants under one year old with acute hepatic failure and severe encephalopathy.
- This approach can improve outcomes for critically ill pediatric patients awaiting liver transplantation.
- MARS offers a potential life-saving intervention in pediatric acute liver failure, facilitating successful transplantation and recovery.
Background:
Acute liver failure (ALF) in children is a rare but often fatal event. At present, liver transplantation is the only successful therapy in most cases. In the face of deteriorating hepatic encephalopathy in these children, some bridging therapy using artificial detoxification can be necessary to enable successful transplantation. In adults, albumin dialysis using the molecular absorbent recycling system (MARS) has been described as effective for bridging to liver transplantation.
Case Report:
A previously healthy 7-month-old infant was admitted with ALF due to autoimmune hepatitis. King's College criteria for children with ALF indicated the need for transplantation (bilirubin 13.7 mg/dl, leukocytes 18,980/mm3, INR 5.83, age<2 years). Despite moderate hyperammonemia (75 microm/l) along with the development of pneumonia, the child deteriorated hemodynamically and neurologically, showing grade III encephalopathy proven by EEG. Albumin dialysis using MARS was used to bridge 36 hours to successful living-donor split-liver transplantation, and resulted in improvements in EEG, plasma levels of amino acids and hemodynamics. Twenty-four months after transplantation the child shows normal liver function and normal neuropsychological development. The explanted liver showed 80 % tissue destruction from autoimmune hepatitis.
Conclusion:
Albumin dialysis as described can be used successfully in infants < 1 year old for bridging to liver transplantation in cases of acute hepatic failure with deteriorating encephalopathy.
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