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Generation of a Rat Model of Acute Liver Failure by Combining 70% Partial Hepatectomy and Acetaminophen
Published on: November 27, 2019
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Phenotype and Long-Term Outcome in Recurrent Paediatric Acute Liver Failure: Systematic Review and Individual
Harry Sutton1, Or Steg Saban1, Jessie Cunningham2
1Division of Gastroenterology Hepatology and Nutrition, Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.
Summary
Recurrent acute liver failure (RALF) in children, often linked to genetic mutations, typically presents in infancy and may resolve spontaneously. Genetic testing for NBAS, SCYL1, RINT1, and LARS1 is recommended for unexplained cases.
Area of Science:
- Pediatric Hepatology
- Clinical Genetics
- Genomic Medicine
Background:
- Recurrent acute liver failure (RALF) in children involves multiple episodes of liver failure with full recovery between events.
- Genetic mutations in NBAS, RINT1, LARS1, and SCYL1 are associated with RALF.
- Understanding transplant and mortality rates is crucial for managing pediatric RALF.
Purpose of the Study:
- To systematically review and analyze individual patient data for RALF in children with specific genetic mutations.
- To determine transplant and mortality rates in this patient population.
- To inform clinical decision-making for managing pediatric RALF.
Main Methods:
- Systematic review and one-stage individual participant analysis.
- Searched EMBASE, MEDLINE, and Web of Science for English-language studies up to May 14th, 2025.
- Included studies on pediatric RALF with NBAS, RINT1, LARS1, or SCYL1 mutations.
Main Results:
- 168 patients with RALF were identified, with a median first presentation at 9 months.
- NBAS (66%) and LARS1 (18.4%) were the most common mutated genes.
- 11% underwent liver transplant, and 17.3% died; no recurrences post-transplant were noted.
Conclusions:
- RALF in children often presents in the first year of life and can have a self-limiting course, frequently triggered by febrile illness.
- Liver transplantation for RALF is controversial and may be less indicated for conditions that resolve with age.
- Whole-exome sequencing targeting NBAS, SCYL1, RINT1, and LARS1 is recommended for unexplained acute liver failure in febrile children.
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