Fulminant hepatic failure (FHF)

Pathology:

Severe hepatic failure in which encephalopathy develops in under two weeks in a pt with a previously normal liver

Cases that develop at a slower pace (2-12 weeks) are called subacute or subfulminant hepatic failure

FHF is a rare but often life-threatening syndrome that is due to acute hepatitis from any cause

Histologically, there is multiacinar necrosis involving a substantial part of the liver

Clinical features:

Jaundice

Small liver

Signs of hepatic encephalopathy

The mental state varies from:

Drowsiness, confusion and disorientation (grades I-II) to

Unresponsive coma (grade IV)

Fetor hepaticus

Fever

Vomiting

Hypotension

Hypoglycaemia

Cerebral oedema – leads to intracranial hypertension and brain herniation (the most common cause of death)

Investigations:

Hyperbilirubinaemia

High serum aminotransferases

Low levels of coagulation factors

EEG to grade the encephalopathy

Treatment:

When ICP is raised, 20% mannitol (1g/kg bodyweight) should be infused IV

Hypoglycaemia, hypokalaemia and hypocalcaemia should be anticipated and corrected

Coagulopathy is managed with IV vitamin K, platelets, blood or FFP

H₂-receptor antagonists to prevent GI bleeding

Infection should be treated with appropriate antibiotics

Flumazenil (a BDZ antagonist) may give a transient improvement in encephalopathy

Liver transplantation

Course and prognosis:

In mild cases (grades I and II encephalopathy with drowsiness and confusion), 66% of pts will survive

The outcome of severe cases (grades III and IV encephalopathy with stupor or deep coma) is related to the aetiology