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Acute (Fulminant) Hepatic Failure (FHF) Syndrome in which hepatic encephalopathy results from a sudden severe impairment of hepatic function
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Acute (Fulminant) Hepatic Failure (FHF) • Syndrome in which hepatic encephalopathy results from a sudden severe impairment of hepatic function • Occurring within 8 weeks of onset of the Acute liver disease, in the absence of evidence of pre-existing liver disease (to distinguish it from deterioration in CLD that also leads to HE) Dr S Chakradhar
Aetiology Any cause of acute liver disease • Acute Viral hepatitis (commonest) • Drugs e.g. parcetamol in overdose , Halothane, MAO inhibitors • Toxins Aflatoxin , CCl4 • Miscellaneous - Wilson’s disease, pregnancy , Reye’s syndrome Shock Dr S Chakradhar
Clinical features 1. Features of cerebral disturbances (Encephalopathy) Dr S Chakradhar
2. Features of Cerebral oedema • Unequal or abnormally reacting pupils, fixed pupils • Hyperventilation, profuse sweating • Local or general myoclonus, focal fits , decerebrate posturing • Papilloedema does not occur or a late sign Dr S Chakradhar
3. Others • Weakness , nausea & vomiting • Jaundice • Fetor hepaticus • Enlarged liver (early), later impalpable • Ascites, edema • Features of complications Dr S Chakradhar
Complications Dr S Chakradhar
Investigations • Blood Leucocytosis • LFT • Prothrombin time - much prolonged • Plasma amino transferase (ALT& AST) – high initially but fall if liver damaged • Serum Albumin – normal unless course is prolonged • Serum Alkaline phosphatase - variable • Urine – bilirubin , urobilinogen Liver biopsy contraindicated Dr S Chakradhar
“Starry-sky” Dr S Chakradhar
Management • Pt should be treated in ICU. Close observation so that complication can be corrected promptly Measures for encephalopathy • See Hepatic Encephalopathy Measures for cerebral oedema • Mannitol 20% (1gm/kg) iv infusion over 30 minutes repeated if necessary Dr S Chakradhar
Nutrition Glucose (300gm/d) orally or into a large central vein as 10 -20 % solution (no protein) Correction of electrolytes • Improvement of circulatory function - I/V fluids N acetylcysteine 150gm/kg stat then 10 mg/kg hourly • Respiratory failure – assisted ventilation • Hemorrhage – inj Vit K, Platelets, blood or plasma. H2 blocker • Infection – cefotaxime or augmentin Liver transplantation in progressive liver failure Dr S Chakradhar
Course & prognosis Mild cases - (grade 1 & 2 encephalopathy with drowsiness & confusion) • 2/3rd of the pt survive Severe cases – grade 3 & 4 encephalopathy with stupor or deep coma • Is related to the aetiology Dr S Chakradhar
Reye's syndrome • This is a rare acute encephalopathy in which cerebral edema & severe fatty degeneration of the liver develops after an infectious illness such as influenza or chicken pox which has been often treated with aspirin • It is common in children & adolescents • Jaundice is not seen Dr S Chakradhar