ArticlePloS one2018
The endothelial specific isoform of type XVIII collagen correlates to annual bleeding rate in haemophilia patients.
Article in PloS one, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 4 papers.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Who cites it
4 citing papers in PubMed, 13 citations in OpenAlex.
- Factor VIII promotes angiogenesis and vessel stability regulating extracellular matrix proteins.Haematologica · 2024Article
- Application of an Extracellular Matrix-Mimicking Fluorescent Polymer for the Detection of Proteolytic Venom Toxins.Toxins · 2023Article
- The Progression of Hemophilic Arthropathy: The Role of Biomarkers.International journal of molecular sciences · 2020Review
- Mechanisms of vascular permeability and remodeling associated with hemarthrosis in factor VIII-deficient mice.Journal of thrombosis and haemostasis : JTH · 2019Article
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Authors and funding
5 authors at 2 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
introductionThe medical need in the haemophilic (HF) field to reduce bleeding incidents requires measurement of the annual bleeding rate (ABR) in haemophiliacs. Vascular rupture is associated with damage to the vascular endothelium causing exposure of the basement membrane. Endothelial cells and matrix impairment may be associated with joint bleeds and later development of HF arthropathy. Imbalanced extracellular matrix turnover is a central pathological feature in many diseases consequent to epithelial or endothelial cell damage. Type XVIII collagen is an essential basement membrane component, with an endothelial specific isoform.
aimTo quantify the basement membrane specifically for the endothelial cells, as that may have particular relevance to endothelial cell stability and rupture in haemophiliacs. A newly developed ELISA assay detecting endothelial type XVIII collagen (COL-18N) was used to assess the clinical relevance of endothelial basement membrane turnover in patients diagnosed with HF arthropathy and correlation to ABR.
methodsWe developed an ELISA assay for quantification of COL-18N. Serum from 35 male HF patients was investigated using the COL-18N ELISA.
resultsCOL-18N correlated to the ABR of haemophiliacs, r = 0.45, P<0.006.
conclusionVascular rupture and consequent bleeding are associated with joint damage and deterioration of life quality in haemophiliacs. Quantification of ABR is an important part in efficacy assessment of different interventions, and the benchmark of these. Objective biomarkers reflecting endothelial dysfunction, vascular leaks and rupture, like the COL-18N biomarker that associate with ABR, may assist in identifying the most optimal treatment and monitoring of HF patients.
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