الفهرس 
EPIDEMIOLOGYOnly 14 pages are availabe for public view
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Abstract
Background: Recurrent haemarthroses is a major complication in haemophilia, contributing to the development of haemophilic (HF) arthropathy. Collagen IV, XV and XVIII are responsible for maintaining vessel wall structure and integrity of the joints basement membranes. Following remodeling and damage, the short isoform of collagen type XVIII is degraded, releasing measurable fragments.Our goal was to quantify the measurable fragments of the endothelial specific isoform of type XVIII collagen (COL-18N) in hemophiliaA patients and to assess its relation to the clinical&radiological data as well as hemophilia joint health score (HJHS), functional independence score for haemophilia (FISH), and hemophilia quality of life.Methods:This cross-sectional study included 50hemophilia A patients recruited from the Pediatric Hematology / Oncology unit, Children’s Hospital, Ain Shams University,Cairo,Egypt.Quantification of collagen type XVIII was done by ELISA. Assessment of joint status clinically using FISH &HJHS scores and radiologically byX-rays& ultrasound was performed. Results:Hemophilia A patients had significantly higher median collagen type XVIII levels compared to the control group.There was no siginificant difference between the inhibitor positive hemophilia A patients and inhibitor negative regarding collagen type XVIII or between the hemophilia A patients on NSAID and those not . but that patients with 2 target joints had significant higher collagen type XVIII compared to those with one or without target joints.there were significant positive correlations between collagen type XVIII level and the total HJHS,hem QOL, the Ultrasound total score and annual bleeding rate.Conclusion: Our results argued the high level of collagen type XVIII in haemophilia A patients with arthropathy and illustrate its benefit as a potential marker for monitoringthe development of hemophilic arthropathies and tailoring the optimal treatment to prevent further joint damage.