Treatment of Wilson's Disease in Acute Liver Failure

When Wilson's disease presents as acute liver failure, the clinical trajectory can deteriorate rapidly. This scenario demands immediate, structured decision-making — including urgent specialist referral and simultaneous pharmacological intervention.

Clinical Scenario

Acute liver failure due to Wilson's disease represents a high-urgency presentation. All such patients require prompt referral to a liver transplant centre. The presence of hepatic encephalopathy determines the immediacy of listing for transplantation, but referral is indicated regardless.

Management Approach (Partial Overview)

The management pathway involves immediate initiation of pharmacotherapy with chelators, alongside evaluation for liver transplantation. Bridging strategies may be employed while transplant candidacy is determined. Liver transplantation is a central component of the treatment pathway in this setting.

Sequencing, bridging selection, prognostic thresholds, and full criteria are in the complete protocol.

Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.1016/j.jhep.2024.11.007

All patients with ALF due to WD should be referred to LT centres (LoE 2, strong recommendation, strong consensus).
Patients with ALF and encephalopathy should be immediately listed for LT (LoE 2, strong recommendation, strong consensus).
Pharmacotherapy with chelators (rapidly increased to full dose) should be started immediately in all patients with ALF due to WD and those listed for LT, to be continued until LT (LoE 3, strong recommendation, consensus).

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