Socha, Piotr;
Janczyk, Wojciech;
Zanetto, Alberto;
Burra, Patrizia;
Czlonkowska, Anna;
Debray, Dominique;
Ferenci, Peter;
... Tsochatzis, Emmanuel; + view all
(2025)
EASL-ERN Clinical Practice Guidelines on Wilson's disease.
Journal of Hepatology
, 82
(4)
pp. 690-728.
10.1016/j.jhep.2024.11.007.
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Tsochatzis_EASL CPG Wilson fulltext_combined - corrected Clean 10_10_2024.pdf - Accepted Version Access restricted to UCL open access staff until 15 March 2026. Download (1MB) |
Abstract
Wilson's disease is an autosomal recessive disorder of copper metabolism which affects the liver, brain and other organs. Diagnosis is based on: clinical features; biochemical tests, including plasma ceruloplasmin concentration, 24-h urinary copper excretion, copper content in the liver; and molecular analysis. Leipzig score and additionally relative exchangeable copper determination are recommended for diagnosis. Pharmacological therapy comprises chelating agents (penicillamine, trientine) and zinc salts, while only chelators are recommended for significant liver disease. Monitoring is based on clinical symptoms, liver tests and copper metabolism (urinary copper excretion, exchangeable copper) to detect poor compliance and over/under-treatment. Acute liver failure is challenging as making a diagnosis is difficult and pharmacological therapy may not be sufficient to save life. Liver transplantation has a well-defined role in Wilsonian acute hepatic failure but may also be considered in neurological disease.
| Type: | Article |
|---|---|
| Title: | EASL-ERN Clinical Practice Guidelines on Wilson's disease |
| Location: | Netherlands |
| DOI: | 10.1016/j.jhep.2024.11.007 |
| Publisher version: | https://doi.org/10.1016/j.jhep.2024.11.007 |
| Language: | English |
| Additional information: | This version is the author accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions. |
| UCL classification: | UCL UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine > Inst for Liver and Digestive Hlth |
| URI: | https://discovery.ucl.ac.uk/id/eprint/10209938 |
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