Acute Liver Failure

Etiology

• Approx 95-100% of patients with ALF worldwide (particularly in the developing world) have viral hepatitis. The leading cause in the Western world is APAP hepatotoxicity, viral hepatitis is <5-10%.
• Acute viral hepatitis - less than 1% of acute HAV or HBV progresses to ALF.
• "seronegative" hepatitis (non- A to E hepatitis) is often presumed to be viral in origin and is the most common presentation otherwise. It is a diagnosis of exclusion.

Acute Viral Hepatitis

• HAV
  • Diagnosis: positive IgM antibodies at the time of hospitalization (false negative results can occur)
• HBV
  • ALF after acute infection, or new immunosuppressive state with reactivation of chronic infecion, or with coinfection with HDV
  • The severity of reaction and ALF is likely due to the host immune response

Budd-Chiari Syndrome

Defined as outflow obstruction to the hepatic veins. Typically related to thrombosis, but can be secondary to tumour invasion or vascular membrane destruction. Affects young adults and women more than older people and men. 5-year survival is 50-80%. Generally requires transplantation to avoid mortality. The vast majority of patients have a thrombophilia (malignancy, MPN, protein C or S deficiency, PCV, lupus anticoagulant, ATIII deficiency, APS, etc.).

Although BCS can present acutely (<8 weeks), it is more common to present over 3-4 months with ascites, abdo pain, hepatomegaly, jaundice, coagulopathy, elevations in AST and AP levels. Portal hypertension can be variable.

Diagnosis is clinical and combined with Doppler ultrasonography of the hepatic veins, and may include liver biopsy (transjugular route most common due to coagulopathy).

Management of BCS

Medical management: anticoagulations and diuretics to control ascites. Consider thrombolysis with recent onset of disease.

Interventional therapies: consider portosytemic shunting procedures to prevent progression (TIPSS).

Ultimately, liver transplantation remains the definitive treatment for patients failing medical and shunting interventions. They usually require anticoagulation in the immediate postoperative period.