Fulminant Liver Failure

Links:

Acute Liver Failure:
Introduction to the Revised American Association for the Study of Liver Diseases Position Paper on Acute Liver Failure 2011 (PDF)

Alcoholic Hepatitis:
Diagnosis and Treatment of Alcohol‐Associated Liver Diseases: 2019 Practice Guidance From the American Association for the Study of Liver Diseases

ACG Clinical Guideline: Alcoholic Liver Disease

Hepatic Encephalopathy:
Hepatic Encephalopathy in Chronic Liver Disease: 2014 Practice Guideline by AASLD and EASL (PDF)

Transplant Evaluation:
Evaluation for Liver Transplantation in Adults: 2013 Practice Guideline by the AASLD and the American Society of Transplantation (PDF)

“Fulminant Hepatic Failure” – severe liver injury, potentially reversible in nature and with onset of hepatic encephalopathy within 8 weeks of the first symptoms in the absence of pre-existing liver disease”

WEST HAVEN ENCEPHALOPATHY SCORE:

0: Minimal- Not clinically apparent. Detected on neuropsychiatric testing

I: Short attention span, mood changes, sleep rhythm disturbances, impaired addition or                                  subtraction

II: Personality change, disorientation for time, dyspraxia, asterixis (negative myoclonus)

III: Somnolence/semi stupor, confusion, gross disorientation

IV: Coma

ETIOLOGIES:

  • Drug (APAP, cocaine, MDMA)
  • Environmental (mushroom poisoning)
  • Viral Hepatitis (A, B, E in particular)
  • Wilson’s Disease
    • Coombs negative hemolytic anemia, ↑bili, ceruloplasmin <20, high urine Cu
  • Budd Chiari
    • US
    • Autoimmune
  • Antinuclear antibody Anti-smooth muscle antibody Anti-liver kidney microsomal
  • Ischemic

WORK-UP

  • General: CBC, CMP, Coags, APAP level, Tox screen, HIV, Lipase, Ammonia, Lactate
  • APAP: low/absent APAP level does not rule out. AST/ALT >3500 strongly suggests APAP

MANAGEMENT

  • Transfer to liver transplantation center
  • Treat underlying etiology
    • APAP (NAC: IV 150mg/kg over 60min, then 50mg/kg over 4 hours, then 100 mg/kg over 16 hours)
    • Amanita poisoning (Silibinin or PCN G 1g/kg/day + NAC)
    • Acute Reactivation HBV (Tenofovir)
    • HSV/VZV (acyclovir 5-10 mg/kg IV Q8)
    • Wilson’s (albumin dialysis, plasmapheresis/exchange, rare penicillamine)
    • Autoimmune (prednisolone 40-60mg/day)
  • Elevated INR
    • Vitamin K Challenge (10mg IV x1 then 10mg daily SQ)
    • FFP (if active bleeding or invasive procedures) Cryo if fibrinogen <100
  • Thrombocyotopenia
    • Transfuse if active bleeding or invasive procedures
  • Encephalopathy (i.e. cerebral edema) TREAT EARLY AND AGGRESSIVELY
    • Lactulose: goal is 3-4 soft BMs/day
    • Monitor for elevated ICP with optic nerve ultrasound (Goal <20)
    • HOB 30°
    • Goal Na 145-155 (consider 3%NaCl)
    • Avoid drastic changes in CO2, glucose, and fluids (CVVH for RRT)
  • If any sign of infection, give antibiotics early
  • Grade III/IV HE:
    • Intubate (cisatracurium for paralysis if needed, propofol for sedation)
  • Continuous EEG (benzo’s for seizures)
  • Shock (norepinephrine, vasopressin, consider steroids)
  • Nutrition (prone to hypoglycemia, give at least trophic feeds)
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