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Fulminant Hepatitis

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Fulminant Hepatitis, Acute Hepatic Failure, Acute Liver Failure

  • Definition
  1. Rapid onset and progression within weeks to liver necrosis with secondary Hepatic Encephalopathy and Coagulopathy
  • Epidemiology
  1. Incidence: 2000 cases per year in the United States
  • Pathophysiology
  1. Massive hepatic necrosis over the course of days to weeks
  2. Results in rapid progression from Jaundice to encephalopathy and Coagulopathy
  3. Multiorgan failure including Acute Renal Failure follows
  • Types
  1. Timing based on pregression from Jaundice onset to encephalopathy
  2. Acute Liver Failure within 1 week
    1. Hyperacute liver failure
  3. Acute Liver Failure within 1-4 weeks
    1. Acute Liver Failure
  4. Acute Liver Failure over >5-8 weeks
    1. Subacute liver failure
  • Causes
  1. Infectious Disease
    1. Viral Hepatitis
      1. Hepatitis A
      2. Hepatitis B
      3. Hepatitis C
      4. Hepatitis D
    2. Bacterial Infection
    3. Rickettsial infection
    4. Parasitic Infection
  2. Toxic Hepatitis
    1. Hepatotoxin exposure or other drug-induced cause
    2. Examples: Acetaminophen Overdose, Amanita muscaria ingestion
  3. Ischemia or shock
  4. Budd-Chiari Syndrome
  5. Idiopathic Chronic Active Hepatitis
  6. Wilson's Disease (Acute)
  7. Microvesicular Steatosis (Fat) Syndromes
    1. Nonalcoholic Fatty Liver
    2. Acute Fatty Liver of Pregnancy
    3. Reye's Syndrome
  • Symptoms
  1. Vomiting
  2. Upper Abdominal Pain
  3. Anorexia
  4. Jaundice
  • Signs
  1. Neurologic changes (Hepatic Encephalopathy)
    1. Altered Level of Consciousness (Delirium, coma)
    2. Decerebrate rigidity (with severe cerebral edema)
    3. Personality change
  2. Jaundice
  3. Coagulopathy
    1. Bleeding (e.g. Gastrointestinal Bleeding)
  4. Acute Renal Failure (Hepatorenal Syndrome)
  5. Hypoglycemia
  6. Acute Pancreatitis
  7. Cardiopulmonary failure
  8. Ascites (due to Portal Hypertension)
  • Prognosis
  • Factors associated with poor outcomes
  1. Advanced age
  2. Halothane exposure
  3. Hepatitis C
  4. Coma (80% Mortality)
  5. Rapid decrease in liver span
  6. Respiratory Failure
  7. Marked ProTime prolongation
  8. Factor V Level <20%
  • Management
  1. Targeted therapy
    1. Delivery for pregnancy related Acute Liver Disease (especially Acute Fatty Liver of Pregnancy)
    2. Withdraw all known Hepatotoxins
    3. Treat known Hepatotoxin exposures
      1. Consult with poison control and hepatology
      2. Example: N-Acetylcysteine for Acetaminophen Overdose or Amanita muscaria ingestion
  2. Supportive care
    1. ABC Management
      1. Endotracheal Intubation often required
    2. Fluid and electrolytes
      1. Volume expansion with crystalloid initially, but avoid Fluid Overload
      2. Correct acid-base status and electrolyte abnormalities
      3. Monitor Serum Glucose
        1. Correct Hypoglycemia with IV D10 or D20 prn
    3. Prevent GI Bleed
      1. H2 Blockers to maintain gastric pH >3.5
    4. Monitor for infection
      1. Complicated by Bacterial or fungal infection in 80% of cases
      2. Infection is often occult with non-specific changes in status (e.g. worsening encephalopathy)
      3. Routinely monitor urine, Chest XRay and other markers of infection
      4. Consider prophylactic antibiotics
    5. Other measures
      1. Control systemic Hypertension
      2. Consider evaluation of Intracranial Pressure (questionable efficacy)
        1. Lower Intracranial Pressure with Mannitol IV prn
  3. Liver Transplant Indications
    1. Grade 3-4 Encephalopathy
    2. Adverse prognostic indicators as above
  • References
  1. Swencki (2015) Crit Dec Emerg Med 29(11):2-10