Sections View Full Chapter Figures Tables Videos Annotate Full Chapter Figures Tables Videos Supplementary Content + • Acute hepatic injury causes sudden loss in hepatocytes due to toxins, ischemia, or inflammatory reaction to liver• Fulminant hepatic failure defined as onset of encephalopathy within 8 weeks (9-24 weeks for subfulminant) after onset of acute hepatocellular injury• Etiologies include: -Hepatitis viruses-Cytomegalovirus (CMV)-Epstein-Barr virus (EBV)-Varicella-Herpesvirus-Toxins (acetaminophen, isoniazid most common)-Ischemia-Fatty liver of pregnancy-Reye syndrome-Wilson disease-Lymphoma-Hereditary metabolic disorders +++ Epidemiology + • 2000 cases of fulminant or subfulminant hepatic failure annually in United States with 80% mortality +++ Symptoms and Signs + • Jaundice• Right upper quadrant pain• Bleeding• Encephalopathy• Hypotension• Sepsis• Renal failure• Uncal herniation• Corneal rings +++ Laboratory Findings + • Hyperbilirubinemia• Elevated transaminases• Prolonged prothrombin time (PT)• Elevated creatinine +++ Imaging Findings + • Head CT can show cerebral edema + • History of chronic liver disorder• Family history of liver failure (eg, Wilson disease)• Exposure to toxins or drugs• Rapidity of encephalopathy onset + • History and physical exam• Serum antibodies for hepatitis viruses, CMV, and EBV• Ceruloplasmin level (Wilson disease)• Head CT if grade IV encephalopathy present (coma)• Temperature +++ When to Admit + • Any patient with acute hepatic injury, ICU for fulminant cases +++ When to Refer + • Transplantation center whenever encephalopathy develops +++ Surgery + • Liver transplantation• Ventriculostomy for grade IV encephalopathy +++ Indications + • Fulminant or subfulminant failure unresponsive to medical management +++ Contraindications + • Medical comorbidities precluding transplantation, active malignancy +++ Medications + • N-acetylcysteine• Minimizing hypoglycemia• Broad-spectrum antibiotics for any fever (avoid aminoglycosides)• Fresh frozen plasma for planned invasive interventions• Mannitol for elevated intracranial pressure• Vasopressor support for hypotension• Mechanical ventilation for grade IV encephalopathy +++ Treatment Monitoring + • Frequent neurologic exams and head CT scans when indicated• Liver function tests• PT +++ Complications + • Primary nonfunction• Rejection• Biliary leak or stricture• hemorrhage• Hepatic artery thrombosis +++ Prognosis + • 60-70% 5-year survival following liver transplant• All patients recover from acute illness in absence of encephalopathy• Greater than 50% recovery for patients with grade III encephalopathy• Overall survival correlated with grade of encephalopathy• Rapid onset associated with more favorable prognosis• Associated sepsis, acidosis, age < 2 or > 40 years, renal failure, PT > 50 all associated with worse prognosis +++ References ++Schiodt FV, Lee WM. Fulminant liver disease. ... Your Access profile is currently affiliated with '[InstitutionA]' and is in the process of switching affiliations to '[InstitutionB]'. Please click ‘Continue’ to continue the affiliation switch, otherwise click ‘Cancel’ to cancel signing in. Get Free Access Through Your Institution Learn how to see if your library subscribes to McGraw Hill Medical products. Subscribe: Institutional or Individual Sign In Username Error: Please enter User Name Password Error: Please enter Password Forgot Username? Forgot Password? Sign in via OpenAthens Sign in via Shibboleth