Liver transplantation in acute liver failure caused by metabolic diseases: indications and contraindications
This webinar will be on December 15, 2026 (16:00 CET)
Acute liver failure (ALF) caused by metabolic disease is uncommon, rapidly evolving, and challenging in children, where encephalopathy may be absent, late, or difficult to assess. This webinar will present a practical framework for early recognition, emergency stabilization, parallel metabolic and genomic diagnostics, and timely referral to a transplant centre. The central question is not only whether liver transplantation can rescue the patient from ALF, but whether the transplanted liver will correct the lethal metabolic defect without exposing the child to futile surgery.
Wilson disease remains the paradigmatic transplantable metabolic cause of ALF. Massive hepatocellular injury with Coombs-negative hemolysis, renal dysfunction, severe coagulopathy, and characteristic biochemical clues should prompt immediate listing assessment. Encephalopathy, progressive multiorgan deterioration, or unfavourable Wilson-specific prognostic scores support urgent transplantation, while plasma exchange and copper-directed therapy may serve as bridges rather than substitutes for definitive treatment.
Mitochondrial hepatopathies require a more restrictive approach. Transplantation may be considered when liver failure is the dominant, potentially liver-limited manifestation, particularly in selected mtDNA depletion or nuclear gene disorders without advanced neurologic, cardiac, or muscular disease. It is usually contraindicated in generalized mitochondrial cytopathy, Alpers/POLG-related disease, severe neurodevelopmental regression, refractory seizures, progressive cardiomyopathy, uncontrolled sepsis, or irreversible multiorgan failure. Rapid sequencing, lactate trends, neuroimaging, echocardiography, and family counselling are therefore essential before listing.
Hyperammonemia will be discussed as both a consequence of ALF and a primary metabolic emergency, especially in urea cycle disorders. In neonatal or recurrent hyperammonemic crises, transplantation is not primarily an acute detoxification procedure; dialysis, ammonia scavengers, nitrogen restriction, and reversal of catabolism come first. However, elective or urgent transplantation may prevent recurrent life-threatening episodes when the hepatic enzyme defect is transplant-correctable and neurological injury remains limited. Contraindications include irreversible brain damage, uncontrolled infection, refractory shock, or disorders in which liver replacement cannot meaningfully alter prognosis.
Speakers:
·Prof. Piotr Socha, Prof. Piotr Socha, Prof, PhD Department of Gastroenterology, Hepatology and Nutrition, CHMI, Warsaw
·Prof Dariusz Rokicki, associate professor PhD, Department of Pediatrics, Nutrition, and Metabolic Disorders
·Prof Piotr Kaliciński, Prof, PhD Department of Pediatric Surgery and Organ Transplantation, CHMI, Warsaw
Learning outcomes: after attending the webinar, participants will be expected to:
Knowledge:
Recognize metabolic causes of pediatric acute liver failure, especially Wilson disease, mitochondrial hepatopathies, and hyperammonemic disorders, and distinguish transplant-correctable conditions from systemic diseases in which transplantation may be futile.
Practical skills:
Apply a structured emergency approach including metabolic screening, interpretation of ammonia, lactate, hemolysis and liver function tests, early transplant referral, use of bridging therapies, and identification of major contraindications such as irreversible neurological injury, uncontrolled infection, or severe extrahepatic disease.
Professional development:
Improve multidisciplinary decision-making and communication with families regarding prognosis, urgency, transplant risks, and ethical limits of liver transplantation in critically ill children.