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Wilson disease with acute liver failure case presentation
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Wilson disease with acute liver failure case presentation

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  • 1. Acute liver failure Chirag ShahDepartment of Gastroenterology KEM Hospital
  • 2. History• YAS 30-year-old married woman• Referred with – Jaundice since 10 days – No prodromal symptoms – Altered sensorium since 3 days • Progressive • At admission – not responding to verbal commands
  • 3. History• No history of – fever, pain in abdomen, nausea, vomiting – mucosal bleeding, petechiae, ecchymosis – jaundice, ascites, altered sensorium or GI bleed in past• Past history – fracture forearm at 15 years of age; underwent surgery – No history of blood transfusion, tattooing, dental treatment or sexual promiscuity• No history of addiction, recent drug ingestion
  • 4. History• LMP - 15 days back• G2P2A0• Birth history – Third degree consanguinity
  • 5. Examination• Altered sensorium – Not following verbal commands• Temperature – normal• Heart rate 62/min• Respiratory rate 16/min, regular• Icterus +• Pallor +• No clubbing, cyanosis, lymphadenopathy, neck vein engorgement, pedal edema
  • 6. Abdominal examination• Liver – 2 cm palpable, firm with sharp edge, smooth surface• Spleen – not palpable• No flank dullness• No lump• No dilated veins
  • 7. CNS examination• Altered sensorium• GCS – 8/15• No focal neurological deficit• DTR – brisk• Plantar reflex – equivocal• Pupils semi-dilated – reacting to light
  • 8. Clinical diagnosis• Acute liver failure• Etiology ? – Viral hepatitis • Hepatitis E • Hepatitis B • Hepatitis A • Non A non E – Wilson’s disease – Autoimmune hepatitis
  • 9. Investigations• Hemoglobin 11.2 gm/dL• Total count 16000/cumm• Platelet count 1.6 lac/dL• S. Na 142 mEq/L• S. K 4.2 mEq/L• S Creatinine 1.6 mg/dl• BUN 30 mg/dL• S. Ammonia 205 ng/L
  • 10. Liver function testLiver function test 15-12-11 day 5 of illness 20-12-11 day 10Total bilirubin (mg/dL) 13.2 14.8Direct bilirubin (mg/dL) 8.2 8.6AST (IU/mL) 868 900ALT (IU/mL) 375 452Alkaline phosphatase 10 16(IU/mL)Total protein (g/dL) 7.4 7.3Albumin (g/dL) 2.8 2.6INR 4.2 4.5GGT (IU/l) 27 25
  • 11. Investigations• Serology for hepatitis viruses – HbsAg: Negative – Ig M anti HBc antibody: Negative – Anti HCV: Negative – Ig M HEV: Negative – Ig M HAV: Negative
  • 12. Investigations• S. ANA : Negative• S. cerruloplasmin: 8 mg/dL (Normal value:20- 50mg/dl )• 24 hour urinary copper: 110 mcg/24 h• KF ring : Negative
  • 13. Investigations• USG abdomen – Mild hepatomegaly with altered echotexture – Mild splenomegaly – Mild ascites• Hepatoportal doppler – Hepatic veins patent – IVC patent – Portal vein 10 mm – No collaterals
  • 14. Course during hospitalization• Patient deteriorated neurologically• Now, on ventilatory support• Requires transplant• But not affording ……

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