ArticleLiver international : official journal of the International Association for the Study of the Liver2025
Relative Exchangeable Copper, Exchangeable Copper and Total Copper in the Diagnosis of Wilson Disease.
Article in Liver international : official journal of the International Association for the Study of the Liver, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 9 papers.
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Who cites it
9 citing papers in PubMed.
- Genetic testing and exchangeable copper can improve the accuracy of Wilson disease diagnoses.Hepatology communications · 2026Article
- Diagnosing Wilson Disease in Acute Liver Failure: Comparison of Existing and Experimental Biomarkers.The American journal of gastroenterology · 2026Article
- Relative Exchangeable and Exchangeable Copper: Emerging New Biomarkers for Diagnosis and Therapy Monitoring in Wilson's Disease.Liver international : official journal of the International Association for the Study of the Liver · 2026Article
- Repurposing melatonin's therapeutic potential in Wilson disease: Addressing copper overload and redox imbalance.Redox biology · 2026Article
- Identification of age-specific urinary metabolic biomarkers in Wilson disease using machine learning: a comparative study of ensemble tree models.Open medicine (Warsaw, Poland) · 2026Article
- Management of Wilson disease across Europe: an international physician-oriented survey by the ERN-RARE Liver group.Orphanet journal of rare diseases · 2025Article
- Relative exchangeable copper: A highly specific and sensitive biomarker for Wilson disease diagnosis.JHEP reports : innovation in hepatology · 2025Article
- Comparative review of copper-associated chronic hepatitis in dogs and Wilson disease in humans.Frontiers in veterinary science · 2025Review
- The role of copper dysregulation in Wilson disease: an expert opinion.Frontiers in medicine · 2025Review
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Authors and funding
17 authors.
Funding
Abstract
BACKGROUND AND
aimsDiagnosing Wilson disease (WD) remains challenging. The exchangeable copper (CuEXC) methodology measures the non-ceruloplasmin-bound copper fraction in serum. Relative exchangeable copper (REC), the ratio of CuEXC to total serum copper (Total Cu), has been proposed as a potential diagnostic biomarker. This study aimed to evaluate the diagnostic performance of these three copper biomarkers in WD.
methodsCuEXC and Total Cu levels were measured in newly diagnosed treatment-naïve patients with WD (n = 13), treated WD (n = 91), non-Wilsonian hepatic disease (n = 206) and non-Wilsonian acute liver failure (n = 22). REC, CuEXC and Total Cu were compared among groups. Receiver-operating characteristic analyses were performed.
resultsMedian REC was significantly elevated among patients with WD compared to all other groups combined (23.6% vs. 4.9%, p < 0.001). The opposite was found for Total Cu (3.5 μmol/L vs. 17.2 μmol/L, p < 0.001). In newly diagnosed patients with WD, median REC was significantly higher than in treated patients (29.1% vs. 21.6%, p = 0.008). The optimal diagnostic cut-off value for REC was ≥ 13.8% (sensitivity 100% and specificity 99.6%) for newly diagnosed patients versus those with non-Wilsonian hepatic disease. For Total Cu, the optimal cut-off was ≤ 7.1 μmol/L (sensitivity 61.5% and specificity 99.1%) for newly diagnosed patients with WD versus those with non-Wilsonian hepatic disease.
conclusionOur data support the diagnostic value of REC in WD. The more broadly available Total Cu also demonstrates a strong diagnostic performance and may be useful in initial work-up. We suggest including REC and/or Total Cu in a future revision of the Leipzig score.
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