Updated: 12/13/2021

Fulminant Liver Failure

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  • Snapshot
    • An 8-year-old boy is brought to the emergency room by his babysitter for sudden-onset vomiting. He had been feeling ill with headaches, fevers, and myalgias. Today, his parents gave him some anti-pyretics including acetaminophen and aspirin. When his babysitter arrived, he had progressive nausea with vomiting, diarrhea, and increased restlessness. On exam, he is lethargic with hepatomegaly. A head computed tomography shows cerebral edema and his laboratory evaluation reveals increased ammonia and liver enzyme levels. He is admitted to the intensive care unit for further management. (Reye syndrome)
  • 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
        • hepatitis E virus in pregnant women
      • 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
  • ETIOLOGY
    • Pathogenesis
      • mechanism
        • cerebral edema is secondary to both vasogenic factors (increased cerebral blood flow) and cytotoxic edema (↑ ammonia and glutamine)
        • liver 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
          • the most effective treatment
  • Complications
    • Infections
      • often related to invasive procedures during course of hospital stay
    • Seizures
    • Hemorrhage
    • Acute renal failure

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(M2.GI.15.17) A 32-year-old female visits her primary care physician because of recent changes to her health. She states that two weeks ago, following a trip to India, she began to experience severe fatigue, nausea, and vomiting. On exam, the findings in Figure A are observed. The physician obtains liver function tests which demonstrate significantly elevated AST and ALT. Further testing reveals that the patient is infected with an RNA hepevirus. Which of the following features, if present, would be very concerning?

QID: 104808
FIGURES:

Significant IV drug use

11%

(4/36)

Prior similar infection

0%

(0/36)

Pregnancy

75%

(27/36)

Alcoholism

11%

(4/36)

Smoking

0%

(0/36)

M 8 E

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(M2.GI.14.42) A 23-year-old female presents with a week of malaise, muscle and joint aches, nausea, and headaches. Her history is significant for migraine headaches and gonorrhea/chlamydia treated on multiple occasions. She has not traveled recently, but does admit to daily IV heroin use and occasional unprotected sexual intercourse for drugs or money. Physical exam reveals scleral icterus and hepatomegaly. The patient is admitted for supportive treatment. On day 3 of the hospital stay, the physician on-call notices a change in some of the patient's laboratory values, shown in Figure A. The change in laboratory results are most indicative of which of the following conditions?

QID: 106298
FIGURES:

Progression to chronic hepatitis

23%

(6/26)

Superinfection with hepatitis D on preexisting hepatitis B

4%

(1/26)

Resolution of an acute hepatitis

15%

(4/26)

Alcoholic hepatitis

0%

(0/26)

Progression to fulminant hepatitis

54%

(14/26)

M 6 D

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Evidence (8)
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EXPERT COMMENTS (1)
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