A 22 year old woman is evaluated in the emergency for a 3 day h/o dark urine and abdominal distension. O/E: normal mental status, icterus present, hea and lungs normal, Hematocrit: 26, Reticulocytes: 5%, Platelets: 1.3 lakhs, Alk.Phos: 30 units/L, ALT: 110 units/L, AST: 220 units/L, Total bilirubin:13mg% (Direct:4 mg) HBsAg positive and Hepatitis ‘A’ &’C’ negative. Urine drug screen negative. USG abdomen shows a nodular appearing liver and enlarged spleen. Which is the most likely diagnosis?
Correct Answer: Wilson disease
Description: Points in or of diagnosis of Wilson's disease- Low hematocrit of 26% and increased reticulocyte count: hemolysis of RBC due to excess intracellular copper. Unconjugated jaundice due to hemolysis and defective conjugation in liver due to hepatocyte damage. Mild elevation of enzymes unlike acute viral hepatitis where gross elevations in enzyme values Nodular liver with splenomegaly indicating a chronic process. Poal hypeension has already developed in the patient. Hepatitis B positivity in the question was given to confuse you, since it can be an incidental finding. Points against the diagnosis of acute viral hepatitis Jaundice with mild elevation of AST/ALT Nodular enlarged liver Enlarged spleen ALSO KNOW: A prognostic index for wilson diseasecalculated by Nazer (score range 0-12) based on serum bilirubin, serum AST, and prolongation in prothrombin time. KING'S COLLEGE CRITERIA:(were developed to determine which patients with fulminant hepatic failure (FHF) should be referred for liver transplant.)
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