Acute liver failure

Overview & Definition

A previously well child who develops jaundice, easy bruising, and altered consciousness over days has acute liver failure, one of the most time-critical emergencies in paediatrics. The defining problem is sudden, massive hepatocyte loss that destroys the liver's synthetic and detoxifying functions, producing coagulopathy, encephalopathy, and multiorgan failure that can progress to death within days without liver transplantation. [1]

The paediatric definition differs from the adult one in one crucial respect, and examiners test this repeatedly. In adults, hepatic encephalopathy is required to diagnose acute liver failure. In children, encephalopathy is not required, because infants and young children may not show recognisable encephalopathy until very late, and waiting for it delays life-saving treatment. The Paediatric Acute Liver Failure Study Group definition requires acute liver injury with coagulopathy defined as an international normalised ratio of 1.5 or greater with any degree of hepatic encephalopathy, or an international normalised ratio of 2.0 or greater without encephalopathy, in a child with no evidence of chronic liver disease. [1]

The emergency is two-sided. The failing liver stops making clotting factors and clearing toxins, so the child bleeds and accumulates ammonia that swells the brain. At the same time the cause may be treatable, which is why every minute spent on resuscitation must run in parallel with a hunt for the cause and an early call to the liver transplant centre. The window between irreversible brain injury and a successful transplant is narrow, and the team that recognises failure early and escalates fast saves lives. [5]

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1. [1]Squires JE, Alonso EM, Ibrahim SH, et al. North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition Position Paper on the Diagnosis and Management of Pediatric Acute Liver Failure. J Pediatr Gastroenterol Nutr, 2022.PMID 34347674
2. [2]O'Grady JG, Alexander GJ, Hayllar KM, Williams R Early indicators of prognosis in fulminant hepatic failure. Gastroenterology, 1989.PMID 2490426
3. [3]Lee WM, Hynan LS, Rossaro L, Fontana RJ, Stravitz RT, Larson AM, et al Intravenous N-acetylcysteine improves transplant-free survival in early stage non-acetaminophen acute liver failure. Gastroenterology, 2009.PMID 19524577
4. [4]Kortsalioudaki C, Taylor RM, Cheeseman P, Bansal S, Mieli-Vergani G, Dhawan A Safety and efficacy of N-acetylcysteine in children with non-acetaminophen-induced acute liver failure. Liver Transpl, 2008.PMID 18161828
5. [5]Squires JE, McKiernan P, Squires RH Acute Liver Failure: An Update. Clin Liver Dis, 2018.PMID 30266162
6. [6]Devictor D, Tissieres P, Durand P, Debray D Acute liver failure in neonates, infants and children. Expert Rev Gastroenterol Hepatol, 2011.PMID 22017699
7. [7]Kulkarni SS, Goss CW, Khan AS, et al. Outcomes Analyses of Pediatric Acute Liver Failure Subjects Listed for Liver Transplantation. J Pediatr Gastroenterol Nutr, 2022.PMID 35442235
8. [8]Lal BB, Khanna R, Sood V, et al. Diagnosis and management of pediatric acute liver failure: consensus recommendations of the Indian Society of Pediatric Gastroenterology, Hepatology, and Nutrition (ISPGHAN). Hepatol Int, 2024.PMID 39212863
9. [9]Pamecha V, Patil NS, Falari S, et al. Live donor liver transplantation for pediatric acute liver failure: challenges and outcomes. Hepatol Int, 2023.PMID 37587287
10. [10]Squires RH Jr Acute liver failure in children. Semin Liver Dis, 2008.PMID 18452115
11. [11]Kerkar N, Rana A Wilson Disease in Children. Clin Liver Dis, 2022.PMID 35868686
12. [12]Sorensen LG, Neighbors K, Hardison RM, et al. Health Related Quality of Life and Neurocognitive Outcomes in the First Year after Pediatric Acute Liver Failure. J Pediatr, 2018.PMID 29551316