ArticleOrphanet journal of rare diseases2018
High hepatic macrophage activation and low liver function in stable Wilson patients - a Danish cross-sectional study.
Article in Orphanet journal of rare diseases, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT02702765 (Macrophages and the Macrophage Activation Markers sCD163 and Mannose Receptor), which is not on this map. Cited by 7 papers.
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The trial behind it
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Macrophages and the Macrophage Activation Markers sCD163 and Mannose Receptor (sMR) in Patients With Wilsons Disease - Associations With Liver Disease Severity and Fibrosis
Who cites it
7 citing papers in PubMed, 13 citations in OpenAlex.
- The gut-liver-kidney-brain axis in Wilson disease: copper speciation-flux and barrier-mediated organ crosstalk.Frontiers in immunology · 2026Review
- Biomarker Discovery in Wilson's Disease-A Path Toward Improved Diagnosis and Management: A Comprehensive Review.Molecular neurobiology · 2025Review
- Impact of liver fibrosis on AAV-mediated gene transfer to mouse hepatocytes.Nature communications · 2025Article
- Cognitive impairment in stable Wilson disease across phenotype.Metabolic brain disease · 2021Article
- The macrophage activation marker soluble CD163 is elevated and associated with liver disease phenotype in patients with Wilson's disease.Orphanet journal of rare diseases · 2020Article
- Macrophage Activation Markers, Soluble CD163 and Mannose Receptor, in Liver Fibrosis.Frontiers in medicine · 2020Review
- Kupffer Cells: Important Participant of Hepatic Alveolar Echinococcosis.Frontiers in cellular and infection microbiology · 2020Article
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Authors and funding
7 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundHepatic macrophage (Kupffer cell) hyperplasia is often described in Wilson's disease (WD). In many liver diseases, Kupffer cell activation is related to disease severity, liver function, and fibrosis but the importance in WD is unknown. Kupffer cell activation can be assessed by the P-concentration of soluble (s)CD163, metabolic liver function by the galactose elimination capacity (GEC), and fibrosis by Fibroscan. We investigated the associations between sCD163, selected inflammatory cytokines, GEC, and liver fibrosis in Danish WD patients.
methodsIn a cross-sectional design, we studied 29 stable and well-treated patients (male/female15/14) with a median age of 35 years (IQR 24-50). P-sCD163 and cytokines were measured by ELISA. The GEC was measured by intra-venous galactose loading.
resultsThe median P-sCD163 value at 2.96 mg/L (1.97-3.93) was high in the normal range (0.7-3.9) and seven patients (24%) had a value above the upper normal value. sCD163 correlated with TNF-α, IL-6 and IL-8 (rho> 0.50, p < 0.005). A higher sCD163 value was closely associated with a lower GEC (rho = - 0.51, p = 0.02). sCD163 was not related to the liver fibrosis indices.
conclusionsStable WD patients showed various degrees of Kupffer cell activation which was accompanied by loss of metabolic liver function. Neither activation nor liver function was related to liver fibrosis. The findings suggest that in WD inflammatory Kupffer cell activation may be involved in the loss of liver function over time. sCD163 may serve as a non-invasive biomarker of loss of liver function in WD, which the degree of fibrosis evidently may not. This study is registered at clinical trials with name: "sCD163 and sMR in Wilsons Disease - Associations With Disease Severity and Fibrosis", NCT02702765. Date of registration: 26.02.16. Date of enrolment of the first participant to the trial: 17.03.16. ULR: https://clinicaltrials.gov/ct2/show/NCT02702765 .
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