Abrupt collapse of liver function and hepatic encephalopathy, which is the sine qua non of the syndrome.
Ref2242ed to as fulminant hepatic failure.
Approximately 2000 cases per year in the U.S.
Rare process with 10 cases per million persons per year in the developed world.
Most common in previously healthy adults in their 30s.
Rare, abrupt onset of severe liver injury with profound liver dysfunction and hepatic encephalopathy.
Patients have no known preceding liver disease.
Patients with fulminant hepatic failure have the potential for complete recovery to normal liver function.
Patients present with hepatic dysfunction, abnormal biochemical liver tests, coagulopathy, and encephalopathy may develop with multiorgan failure and death in up to 50% of cases.
Prognosis is dismal but improved with liver transplantation.
About 6% of liver transplants performed on patients with acute liver failure each year.
Shift in poor prognostic lesions from hepatitis B to those with a relatively good prognosis, acetaminophen overdose.
Most cases in adults in the U.S. are due to acetaminophen, with other drugs, viral hepatitis, shock liver and other undefined etiologies account for remaining cases.
Characterized by encephalopathy, coagulopathy and hepatic failure.
May be related to Wilson’s disease and autoimmune hepatitis.
Jaundice that precedes encephalopathy by at least one week indicates a poor prognosis.
Fulminant hepatic failure of greater than 2 week duration associated with high likelihood of portal hypertension and mortality.
Degree of hepatic encephalopathy is strong predictor of survival with grade II encephalopathy associated with a 65-70% chance of survivorship, grade III or IV have a 30-50% and 20% chance of survival, respectively.
King’s College Hospital Criteria for Liver Transplantation:
Acetaminophen induced disease=
Arterial pH <7.30 or prothrombin time> 100 seconds and creatinine >3.4 mg/dL and grades III or IV encephalopathy.
A single factor in acetaminophen induced disease is associated with a mortality rate of 55% and the presence of severe acidosis associated with a 95% mortality.
Nonacetaminphen associated acute liver failure=
Prothrombin time >100 seconds or any three of the following age < 10 years or greater than 40 years, non A, non B viral hepatitis, halothane hepatitis, or idiosyncratic drug reaction, duration of jaundice before the onset of encephalopathy greater than 7 days, prothrombin time greater than 50 seconds, serum bilirubin >18 mg/dL
In nonacetaminophen acute liver failure the presence of a single factor is associated with a mortality rate of 80% and rate of 95% with any three factors.