Fulminant, or acute, hepatic failure is defined as severe hepatocyte dysfunction resulting in rapid elevation of aminotransferases, encephalopathy, coagulopathy and multiorgan failure in an otherwise healthy individual without preexisting liver disease. Acute liver failure (ALF) has an incidence of 1–2/100,000 people in the United States or approximately 3,000–6,000 cases per year with nearly 30% of patients requiring a liver transplantation. ALF is fundamentally different and should not be confused with acute or chronic liver failure or decompensated cirrhosis, as the etiology of ALF is the most important determinant of transplant-free survival.

Key Learning Points

1. 1. Children with acute liver failure (ALF) require rapid stabilisation and should be moved to a specialist centre as quickly as possible.

2. 2. Once on a paediatric intensive care unit, ALF patients undergo standard neurocritical intensive care and a transplant assessment.

3. 3. In ALF, children with encephalopathy more severe than grade 1 should be considered for intubation.

4. 4. The mean arterial pressures targeted for encephalopathic patients vary with age.

5. 5. In diabetic ketoacidosis, cerebral oedema is the most common cause of mortality and neurological observations should be performed regularly.

Key Learning Points

1. 1. The acute-on-chronic liver failure (ACLF) syndrome describes acute decompensation of liver function in the context of chronic liver disease.

2. 2. ACLF is associated with extra-hepatic organ failure and high mortality.

3. 3. Underlying chronic liver disease reflects typical population prevalence; the acute precipitant is often alcohol toxicity, systemic sepsis or viral hepatitis, although up to half of cases have no discernible aetiology.

4. 4. Systemic inflammation and bacterial translocation are major pathophysiological components.

5. 5. At present, there are no evidence-based interventions for ACLF, other than supportive care in the intensive care unit and assessment for urgent liver transplantation.