New EASL-ERN Clinical Practice Guidelines on Wilson disease

We are happy to release the updated Wilson disease guideline which is the results of EASL-ERN RARE-LIVER collaborative work with endorsement of ESPGHAN. The value of the guideline is methodology which strictly followed EASL guidelines how to write guidelines. The update introduced several novelties.

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Differential diagnosis still is a challenge and this was further described in detail including recently described diseases with similar clinical presentation and risk to confuse with AIH was presented based on recent data. The previous scoring system for diagnosis was also accepted but additionaly a new method was added to the system which is REC. Monitoring of therapy was described in detail and included exchangeable copper in addition to previous parametres. Indications for liver transplantation were revisited and neurological indication was added. Management of acute liver failure was described in detail, including pharmacotherapy and plasma exchange. Extensive part of the guidelines covered neurological presentation, including indications for neurological examinations also in liver presentation and in paediatric patients

If you’re interested in reading the full paper, you can access it directly through the publication link.