{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,7]],"date-time":"2026-07-07T17:26:22Z","timestamp":1783445182599,"version":"3.54.6"},"reference-count":41,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2022,5,30]],"date-time":"2022-05-30T00:00:00Z","timestamp":1653868800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Haemophilia"],"published-print":{"date-parts":[[2022,9]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Haemophilic arthropathy (HA) is a major complication in haemophilia. Collagens IV, XV and XVIII are responsible for maintaining the integrity of the vessel wall in the joint. Following joint remodelling and damage, the short isoform of collagen type XVIII (COL\u201018N) is degraded, releasing measurable fragments. Our goal was to quantify the specific isoform COL\u201018N in haemophilia A patients and to assess its relation to the clinical and radiological data as well as haemophilia joint health score (HJHS), functional independence score for haemophilia (FISH), and haemophilia quality of life (Haemo\u2010Qol).<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>This cross\u2010sectional study included 50 haemophilia A patients recruited from the Paediatric Haematology and Oncology unit, Ain Shams University, Cairo, Egypt. Quantification of COL\u201018N was done by ELISA. Assessment of joint state clinically using FISH and HJH scores and radiologically by X\u2010rays and ultrasound.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Haemophilia A patients had significantly higher median COL\u201018N levels compared to the control group. Inhibitor positive and negative haemophilia A patients as well as those on non\u2010steroidal anti\u2010inflammatory drug and those not had comparable COL\u201018N levels. Patients with \u22652 target joints had significantly higher COL\u201018N level compared to those with one or those without target joints. There were significant positive correlations between COL\u201018N level and the total HJHS, Haemo\u2010Qol, the HEAD\u2010US score and annual bleeding rate.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Our results demonstrated a high level of COL\u201018N in haemophilia A patients and argued its benefit as a potential marker for monitoring the development of haemophilic arthropathy and tailoring the optimal treatment to prevent further joint damage.<\/jats:p><\/jats:sec>","DOI":"10.1111\/hae.14593","type":"journal-article","created":{"date-parts":[[2022,5,30]],"date-time":"2022-05-30T22:33:45Z","timestamp":1653950025000},"page":"849-856","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Endothelial specific isoform of type XVIII collagen (COL\u201018N): A marker of vascular integrity in haemophilic arthropathy"],"prefix":"10.1111","volume":"28","author":[{"given":"Azza Abdel Gawad","family":"Tantawy","sequence":"first","affiliation":[{"name":"Pediatric Haematology\/Oncology Childrens Hospital, Ain Shams University  Cairo Egypt"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7249-7971","authenticated-orcid":false,"given":"Nayera H. 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