Acute liver failure (ALF) presenting in the context of suspected or confirmed Wilson disease is a distinct and serious clinical scenario. The underlying copper metabolism disorder shapes the management approach from the outset, requiring prompt evaluation and specific temporizing interventions.
This protocol applies to patients presenting with ALF in whom Wilson disease is suspected or confirmed. The presence of this inherited copper metabolism disorder significantly influences both the urgency of evaluation and the treatment pathway, given the limited effectiveness of standard medical therapy in this setting.
Management in this scenario centres on temporizing measures directed at copper depletion — including extracorporeal approaches — while liver transplantation (LT) evaluation is initiated early in the diagnostic workup, given the lack of effective medical therapy. The complete structured regimen, sequence, and criteria are available in the full protocol.
DOI: 10.14309/ajg.0000000000002340
In patients presenting with ALF due to suspected or confirmed Wilson disease, LT evaluation should be initiated during diagnosis due to the lack of effective medical therapy.
Various interventions such as plasmapheresis, albumin dialysis, plasma exchange, and continuous hemofiltration have been used for copper depletion to avoid renal injury but are only temporizing measures.
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