Updated: 10/8/2019

Fulminant Liver Failure

Topic
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Questions
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Evidence
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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
  • 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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Questions (4)
Lab Values
Blood, Plasma, Serum Reference Range
ALT 8-20 U/L
Amylase, serum 25-125 U/L
AST 8-20 U/L
Bilirubin, serum (adult) Total // Direct 0.1-1.0 mg/dL // 0.0-0.3 mg/dL
Calcium, serum (Ca2+) 8.4-10.2 mg/dL
Cholesterol, serum Rec: < 200 mg/dL
Cortisol, serum 0800 h: 5-23 μg/dL //1600 h:
3-15 μg/dL
2000 h: ≤ 50% of 0800 h
Creatine kinase, serum Male: 25-90 U/L
Female: 10-70 U/L
Creatinine, serum 0.6-1.2 mg/dL
Electrolytes, serum  
Sodium (Na+) 136-145 mEq/L
Chloride (Cl-) 95-105 mEq/L
Potassium (K+) 3.5-5.0 mEq/L
Bicarbonate (HCO3-) 22-28 mEq/L
Magnesium (Mg2+) 1.5-2.0 mEq/L
Estriol, total, serum (in pregnancy)  
24-28 wks // 32-36 wks 30-170 ng/mL // 60-280 ng/mL
28-32 wk // 36-40 wks 40-220 ng/mL // 80-350 ng/mL
Ferritin, serum Male: 15-200 ng/mL
Female: 12-150 ng/mL
Follicle-stimulating hormone, serum/plasma Male: 4-25 mIU/mL
Female: premenopause: 4-30 mIU/mL
midcycle peak: 10-90 mIU/mL
postmenopause: 40-250
pH 7.35-7.45
PCO2 33-45 mmHg
PO2 75-105 mmHg
Glucose, serum Fasting: 70-110 mg/dL
2-h postprandial:<120 mg/dL
Growth hormone - arginine stimulation Fasting: <5 ng/mL
Provocative stimuli: > 7ng/mL
Immunoglobulins, serum  
IgA 76-390 mg/dL
IgE 0-380 IU/mL
IgG 650-1500 mg/dL
IgM 40-345 mg/dL
Iron 50-170 μg/dL
Lactate dehydrogenase, serum 45-90 U/L
Luteinizing hormone, serum/plasma Male: 6-23 mIU/mL
Female: follicular phase: 5-30 mIU/mL
midcycle: 75-150 mIU/mL
postmenopause 30-200 mIU/mL
Osmolality, serum 275-295 mOsmol/kd H2O
Parathyroid hormone, serume, N-terminal 230-630 pg/mL
Phosphatase (alkaline), serum (p-NPP at 30° C) 20-70 U/L
Phosphorus (inorganic), serum 3.0-4.5 mg/dL
Prolactin, serum (hPRL) < 20 ng/mL
Proteins, serum  
Total (recumbent) 6.0-7.8 g/dL
Albumin 3.5-5.5 g/dL
Globulin 2.3-3.5 g/dL
Thyroid-stimulating hormone, serum or plasma .5-5.0 μU/mL
Thyroidal iodine (123I) uptake 8%-30% of administered dose/24h
Thyroxine (T4), serum 5-12 μg/dL
Triglycerides, serum 35-160 mg/dL
Triiodothyronine (T3), serum (RIA) 115-190 ng/dL
Triiodothyronine (T3) resin uptake 25%-35%
Urea nitrogen, serum 7-18 mg/dL
Uric acid, serum 3.0-8.2 mg/dL
Hematologic Reference Range
Bleeding time 2-7 minutes
Erythrocyte count Male: 4.3-5.9 million/mm3
Female: 3.5-5.5 million mm3
Erythrocyte sedimentation rate (Westergren) Male: 0-15 mm/h
Female: 0-20 mm/h
Hematocrit Male: 41%-53%
Female: 36%-46%
Hemoglobin A1c ≤ 6 %
Hemoglobin, blood Male: 13.5-17.5 g/dL
Female: 12.0-16.0 g/dL
Hemoglobin, plasma 1-4 mg/dL
Leukocyte count and differential  
Leukocyte count 4,500-11,000/mm3
Segmented neutrophils 54%-62%
Bands 3%-5%
Eosinophils 1%-3%
Basophils 0%-0.75%
Lymphocytes 25%-33%
Monocytes 3%-7%
Mean corpuscular hemoglobin 25.4-34.6 pg/cell
Mean corpuscular hemoglobin concentration 31%-36% Hb/cell
Mean corpuscular volume 80-100 μm3
Partial thromboplastin time (activated) 25-40 seconds
Platelet count 150,000-400,000/mm3
Prothrombin time 11-15 seconds
Reticulocyte count 0.5%-1.5% of red cells
Thrombin time < 2 seconds deviation from control
Volume  
Plasma Male: 25-43 mL/kg
Female: 28-45 mL/kg
Red cell Male: 20-36 mL/kg
Female: 19-31 mL/kg
Cerebrospinal Fluid Reference Range
Cell count 0-5/mm3
Chloride 118-132 mEq/L
Gamma globulin 3%-12% total proteins
Glucose 40-70 mg/dL
Pressure 70-180 mm H2O
Proteins, total < 40 mg/dL
Sweat Reference Range
Chloride 0-35 mmol/L
Urine  
Calcium 100-300 mg/24 h
Chloride Varies with intake
Creatinine clearance Male: 97-137 mL/min
Female: 88-128 mL/min
Estriol, total (in pregnancy)  
30 wks 6-18 mg/24 h
35 wks 9-28 mg/24 h
40 wks 13-42 mg/24 h
17-Hydroxycorticosteroids Male: 3.0-10.0 mg/24 h
Female: 2.0-8.0 mg/24 h
17-Ketosteroids, total Male: 8-20 mg/24 h
Female: 6-15 mg/24 h
Osmolality 50-1400 mOsmol/kg H2O
Oxalate 8-40 μg/mL
Potassium Varies with diet
Proteins, total < 150 mg/24 h
Sodium Varies with diet
Uric acid Varies with diet
Body Mass Index (BMI) Adult: 19-25 kg/m2
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(M2.GI.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? Review Topic

QID: 106298
FIGURES:
1

Progression to chronic hepatitis

22%

(5/23)

2

Superinfection with hepatitis D on preexisting hepatitis B

0%

(0/23)

3

Resolution of an acute hepatitis

13%

(3/23)

4

Alcoholic hepatitis

0%

(0/23)

5

Progression to fulminant hepatitis

61%

(14/23)

M2

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SUBMIT RESPONSE 5

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(M2.GI.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? Review Topic

QID: 104808
FIGURES:
1

Significant IV drug use

10%

(3/31)

2

Prior similar infection

0%

(0/31)

3

Pregnancy

74%

(23/31)

4

Alcoholism

13%

(4/31)

5

Smoking

0%

(0/31)

M2

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SUBMIT RESPONSE 3
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