Updated: 10/8/2019

Fulminant Liver Failure

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Snapshot
  • A 16-year-old girl is brought to the emergency room by her parents, who found a half-empty bottle of acetaminophen in her bathroom. She told her parents that she had been feeling depressed and took the pills this morning. Since then, she has had increased abdominal pain, generalized fatigue, and some nausea and vomiting. On physical exam, she is noted to be lethargic with right upper quadrant pain. On laboratory examination, she has elevated liver enzymes as well as elevated acetaminophen levels. She is admitted to the intensive care unit for close monitoring and started on N-acetylcysteine. (Acetaminophen overdose)
Introduction
  • Overview
    • liver failure results in coagulopathy and encephalopathy
      • fulminant liver failure describes onset of encephalopathy within 8 weeks of hepatic injury in a previously healthy patient
  • Epidemiology
    • risk factors
      • drug-induced (most common)
        • acetaminophen
      • Wilson disease
      • viral hepatitis
      • autoimmune hepatitis
      • Reye syndrome
      • HELLP syndrome
      • herbal and dietary supplements
        • ginseng, germander tea, kawakawa, and Teucrium polium
      • certain mushroom ingestions
        • Amanita phalloides mushroom
      • alcohol use
  • Pathogenesis
    • mechanism  
      • cerebral edema is secondary to both vasogenic factors (increased cerebral blood flow) and cytotoxic edema (↑ ammonia and glutamine)
      • live failure is often due to direct toxic effects (i.e., acetaminophen metabolite toxicity)
Presentation
  • Symptoms
    • common symptoms
      • encephalopathy
      • abdominal pain
        • right upper quadrant tenderness present but not always
      • gastrointestinal bleeding
        • melena
        • hematemesis
  • Physical exam
    • inspection
      • jaundice
      • ascites
      • papilledema
        • due to increased intracranial pressure
    • motion
      • hepatomegaly
Imaging
  • Hepatic ultrasound
    •  indications
      • help establish cause of liver failure
    • findings
      • ascites
      • occlusion or patency of vessels
      • liver mass
  • Abdominal computed tomography (CT)
    • indications
      • exclude other intra-abdominal pathologies
    • findings
      • liver mass
      • ascites
      • hepatomegaly
  • Head CT
    • indications
      • exclude other causes of altered mental status
    • findings
      • cerebral edema
Studies
  • Serum labs
    • elevated prothrombin time/INR
      • used to determine severity of coagulopathy
    • ↓ platelets
    • liver panel abnormalities
      • ↑ aspartate aminotransferase (AST)
      • ↑ alanine aminotransferase (ALT)
      • ↑ alkaline phosphatase
      • ↑ bilirubin
    • ↑ ammonia
    • etiology-specific tests
      • autoimmune hepatitis antibodies (ANA and anti-smooth muscle antibody)
      • acetaminophen levels
      • drug screen
      • serum free copper
      • hepatitis viral panels
  • Invasive studies
    • liver biopsy
      • contraindicated in coagulopathy
      • can confirm diagnosis, including autoimmune hepatitis, malignancy, or viral hepatitis
Differential
  • Septic shock
    • key distinguishing factor
      • can also result in multi-organ failure
      • typically does not have the laboratory changes seen in liver failure
 Treatment
  • Management approach
    • address underlying cause (see individual topics for comprehensive review of treatment)
      • i.e. N-acetylcysteine for acetaminophen toxicity
  • Medical
    • supportive care
      • modalities
        • intracranial pressure monitoring
    • treatment of cerebral edema treatment
      • modalities
        • mannitol
    • treatment of coagulopathy
      • modalities
        • fresh frozen plasma
        • recombinant factor VIIa
        • platelet transfusion
  • Surgical
    • liver transplant
      • indications
        • irreversible liver damage
        • most effective therapy
Complications
  • Infections
    • often related to invasive procedures during course of hospital stay
  • Seizures
  • Hemorrhage
  • Acute renal failure
 

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