Describe the fulminant Hepatic Failure of Acute Liver Failure.

Definition

• Acute (fulminant) liver failure is severe acute liver injury with coagulopathy (INR ≥ 1.5 with encephalopathy, or ≥ 2.0 without) in a child without known chronic liver disease.
• It is a life-threatening emergency.

Causes

• Infective — viral hepatitis (A and E common in India; also B).
• Drugs/toxins — paracetamol overdose, anti-tubercular drugs, valproate.
• Metabolic — Wilson disease, galactosaemia, tyrosinaemia (age-dependent).
• Autoimmune hepatitis; ischaemia; indeterminate.

Clinical Features

• Deepening jaundice with hepatic encephalopathy (altered behaviour, drowsiness, coma).
• Coagulopathy (bleeding), hypoglycaemia, and a shrinking liver.
• Complications — cerebral oedema, sepsis, acute kidney injury, multi-organ failure.

Management

• Intensive supportive care — treat/prevent hypoglycaemia (IV dextrose), correct coagulopathy (vitamin K, plasma for bleeding), manage cerebral oedema.
• N-acetylcysteine (for paracetamol and often empirically); specific therapy for the cause (chelation for Wilson, etc.).
• Monitor for and treat sepsis; avoid sedatives/hepatotoxins; protein moderation and lactulose for encephalopathy.
• Early referral for liver transplantation — the definitive treatment for those meeting criteria.

Complications to Anticipate

• Cerebral oedema and raised intracranial pressure (a leading cause of death).
• Hypoglycaemia, coagulopathy and GI bleeding; sepsis; acute kidney injury.
• Electrolyte and acid-base disturbances.

Prognosis & Prognostic Markers

A worsening PT/INR, advancing encephalopathy grade and a shrinking liver indicate a poor prognosis and the need for urgent transplant assessment; the aetiology also matters (paracetamol and hepatitis A do relatively better).

Supportive Monitoring

• Frequent monitoring of glucose, coagulation, electrolytes, ammonia and neurological status.
• Nurse with the head elevated; treat cerebral oedema; maintain a low threshold for antibiotics (sepsis).
• Avoid sedatives and hepatotoxins; correct hypoglycaemia promptly with IV dextrose.