A 28-year-old woman presents with jaundice, Coombs-negative haemolytic anaemia, elevated transaminases (AST 480 U/L), and neuropsychiatric symptoms including dysarthria and tremor. Slit-lamp examination reveals golden-brown rings at the corneal periphery. Serum ceruloplasmin is 8 mg/dL (normal 20–35). The diagnosis is Wilson's disease. Which scoring system helps quantify organ involvement and guide treatment decisions?
- A Leipzig score (Ferenci score) ✓
- B MELD score
- C Child-Pugh score
- D ALFSG prognostic index
Explanation
The Leipzig score (proposed by Ferenci) is the validated diagnostic scoring system for Wilson's disease, incorporating Kayser-Fleischer rings, neuropsychiatric features, Coombs-negative haemolysis, low ceruloplasmin, liver copper, urinary copper and ATP7B mutation analysis; a score ≥4 confirms diagnosis. The MELD and Child-Pugh scores reflect liver synthetic function in established cirrhosis but are not specific to Wilson's. The ALFSG index is used to predict outcomes in acute liver failure. In acute Wilsonian hepatitis with haemolysis and liver failure, liver transplantation is the only curative option if the New Wilson Index (NWI) is ≥11.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
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