Acute Liver Failure In Children

Paediatric acute liver failure is rapid onset severe hepatic dysfunction in a child without known chronic liver disease, characterized by biochemical evidence of liver injury and significant coagulopathy that does not correct with vitamin K. Encephalopathy may occur but is not required for diagnosis in children.

Causes include paracetamol toxicity, viral hepatitis, autoimmune hepatitis, Wilson disease, metabolic disease, mitochondrial disorders, drug toxicity and indeterminate causes.

• Jaundice
• Vomiting
• Abdominal pain
• Hepatomegaly, although the liver may become small in massive necrosis
• Bleeding tendency
• Hypoglycaemia
• Irritability or altered behaviour
• Encephalopathy
• Cerebral oedema in advanced disease

Multiorgan failure may produce shock, acute kidney injury, lactic acidosis and respiratory failure.

Urgently assess:

• ALT and AST
• Bilirubin
• Albumin
• PT and INR
• Glucose
• Electrolytes
• Renal function
• Ammonia
• Lactate and blood gas

Investigate the cause with paracetamol concentration, hepatitis testing, autoimmune markers, ceruloplasmin and copper studies, metabolic investigations and toxicology according to presentation.

Persistent INR at least 1.5 with encephalopathy or at least 2.0 without encephalopathy, not corrected by vitamin K, strongly supports paediatric acute liver failure.

• Transfer early to a centre capable of paediatric intensive care and liver transplantation. Do not wait for severe encephalopathy before discussing transfer.

• Correct hypoglycaemia continuously with glucose containing fluids and frequent glucose monitoring.

• Give vitamin K 0.3 mg/kg IV once, maximum 10 mg, to correct deficiency and clarify whether coagulopathy reflects hepatic synthetic failure.

• Give N acetylcysteine immediately for paracetamol toxicity, using the standard IV protocol. Its routine use in non paracetamol paediatric liver failure is not established and should be specialist directed.

• Do not routinely correct INR with fresh frozen plasma unless there is clinically important bleeding or an invasive procedure is required, because INR is useful for tracking disease severity.

• Manage cerebral oedema, hyperammonaemia, infection, renal failure and haemodynamic instability aggressively in PICU.

• Evaluate for transplantation early when synthetic failure progresses.