Paeds · gastroenterology-hepatology-and-nutrition
Acute liver failure
Also known as Paediatric acute liver failure · PALF · Fulminant hepatic failure in children · Acute hepatic failure · Fulminant hepatitis
Fellowship guide to paediatric acute liver failure: the PALF diagnostic criteria in which encephalopathy is not required unlike adults, the age-dependent aetiology, the use of N-acetylcysteine including for non-acetaminophen causes, the critical point that the adult King's College Criteria are not validated in children, and the early pathway to liver transplant assessment.
On this page
Study tools
Your progress
Saved on this device.
Practise this topic
Target exams
Red flags
- Acute coagulopathy with INR of 1.5 or more and any hepatic encephalopathy, or INR of 2.0 or more without encephalopathy
- Rapidly rising INR, falling arterial pH, or progressive encephalopathy suggesting impending decompensation
- Deepening jaundice with coagulopathy in a previously well child
- Hypoglycaemia, metabolic acidosis, or signs of multiorgan dysfunction
- Suspected acetaminophen ingestion with rising transaminases and falling INR
- Wilson disease presenting as acute liver failure with haemolysis and low alkaline phosphatase
- Neonate with liver failure and signs of galactosaemia, hereditary fructose intolerance, or neonatal haemochromatosis
Life stages
Care settings
Clinical exam formats
Board mappings
- Recognises the paediatric definition of acute liver failure in which encephalopathy is not required
- Identifies the age-dependent causes including indeterminate aetiology, metabolic disease, and drug toxicity
- Initiates resuscitation and urgent referral to a paediatric hepatology and transplant centre
- Applies the PALF diagnostic criteria and distinguishes acute from acute-on-chronic liver failure
- Justifies the use of N-acetylcysteine including for non-acetaminophen causes and explains why King's College Criteria are not validated in children
- Constructs the pathway to liver transplant assessment and listing
- Definition and causes of paediatric acute liver failure by age
- Immediate resuscitation and the role of N-acetylcysteine
- Principles of liver transplant listing in children
- Recognises a deteriorating child with acute liver failure and escalates to intensive care and transplant referral
- Communicates the gravity of acute liver failure and the transplant pathway to a family
- Counsels on prognosis including transplant-free and post-transplant outcomes
- Level 1: Recognition of coagulopathy and hepatic dysfunction as acute liver failure
- Level 2: Initiation of resuscitation, diagnostic workup, and N-acetylcysteine
- Level 3: Coordination of transplant assessment and multidisciplinary intensive care
- Paediatric acute liver failure definition and the absence of an encephalopathy requirement
- Age-dependent aetiology and the role of metabolic and drug-induced causes
- N-acetylcysteine and the limitations of King's College Criteria in children
- Assessment of a child with coagulopathy and hepatic encephalopathy
- Interpretation of liver function and coagulation tests in the emergency setting
- Structured explanation of the resuscitation and transplant pathway
- NASPGHAN approach to diagnosis and management of paediatric acute liver failure
- Acetaminophen toxicity and the role of the paediatric acetylcysteine protocol
- Early transplant centre referral and the limitations of adult prognostic scores
- Recognition of acute liver failure as a time-critical emergency
- Initiation of N-acetylcysteine and urgent specialist referral
- Family communication around transplant and end-of-life decisions
- Canadian approach to paediatric acute liver failure and regional retrieval to transplant centres
- Dynamic prognostic assessment and the move to paediatric-specific listing criteria
- Long-term neurocognitive and quality-of-life follow-up after 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]
References12ShowHide
- [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]O'Grady JG, Alexander GJ, Hayllar KM, Williams R Early indicators of prognosis in fulminant hepatic failure. Gastroenterology, 1989.PMID 2490426
- [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]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]Squires JE, McKiernan P, Squires RH Acute Liver Failure: An Update. Clin Liver Dis, 2018.PMID 30266162
- [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]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]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]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]Squires RH Jr Acute liver failure in children. Semin Liver Dis, 2008.PMID 18452115
- [11]Kerkar N, Rana A Wilson Disease in Children. Clin Liver Dis, 2022.PMID 35868686
- [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