This protocol addresses Wilson's disease presenting with significant hepatic involvement — including liver cirrhosis significant liver fibrosis liver failure or haemolysis. These features indicate active copper-related organ injury requiring a specific first-line treatment approach.
In patients with significant liver disease — whether fibrosis, established cirrhosis, liver failure, or concurrent haemolysis — the choice of treatment strategy is guided by the severity of hepatic involvement. Guidelines specifically direct the first-line approach in this sub-population.
DOI: 10.1016/j.jhep.2024.11.007
Chelators should be the primary choice in patients with significant liver disease, e.g. features of significant fibrosis and cirrhosis, liver failure, and haemolysis (LoE 3, strong recommendation, strong consensus).
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