In acute hepatic decompensation of Wilson's disease presenting with Coombs-negative hemolytic anemia, liver failure, and low ceruloplasmin, which parameter from the Wilson's Index (Nazer score) indicates need for urgent liver transplantation?

• A King Wilson's Index score ≥ 11 ✓
• B Serum bilirubin > 300 µmol/L alone
• C Serum copper > 200 µg/dL
• D AST > 10× ULN

Explanation

The King's Wilson Index (revised Nazer score) is used to predict mortality in acute Wilson's disease and identify patients needing urgent liver transplantation. It incorporates serum bilirubin, AST/ALT, prothrombin time (INR), WBC, and albumin. A score ≥ 11 predicts 100% mortality without liver transplantation. Acute Wilson's disease with Coombs-negative hemolysis reflects massive copper release from the liver. Standard chelation (D-penicillamine, trientine) is insufficient in fulminant disease; plasmapheresis/MARS can be used as a bridge to transplant while calculating the index.

Reference: Harrison's Principles of Internal Medicine, 21st ed.

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Written and medically reviewed by the StethoPrep medical team.