{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,5]],"date-time":"2026-08-05T06:52:34Z","timestamp":1785912754526,"version":"3.56.0"},"reference-count":28,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2021,5,20]],"date-time":"2021-05-20T00:00:00Z","timestamp":1621468800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100000801","name":"Bayer HealthCare","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100000801","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Haemophilia"],"published-print":{"date-parts":[[2021,7]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Objective<\/jats:title><jats:p>The primary objective of this study was to assess whether there are different patterns (classes) of joint health in young boys with severe haemophilia A (SHA) prescribed primary tailored prophylaxis. We also assessed whether age at first index joint bleed, blood group, FVIII gene abnormality variant, factor VIII trough level, first\u2010year bleeding rate and adherence to the prescribed prophylaxis regimen significantly predicted joint damage trajectory, and thus class membership.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Using data collected prospectively as part of the Canadian Hemophilia Primary Prophylaxis Study (CHPS), we implemented a latent class growth mixture model technique to determine how many joint damage classes existed within the cohort. We used a multinomial logistic regression to predict the odds of class membership based on the above predictors. We fitted a survival model to assess whether there were differences in the rate of dose escalation across the groups.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>We identified three distinct classes of trajectory: persistently low, moderately increasing and rapidly increasing joint scores. By multinomial regression, we found that only age at first index joint bleed predicted rapidly increasing joint scores. The rapidly increasing joint score class group moved through dose escalation significantly faster than the other two groups.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Using tailored prophylaxis, boys with SHA follow one of three joint health trajectories. By using knowledge of disease trajectories, clinicians may be able to adjust treatment according to a subject's predicted long\u2010term joint health and institute cost\u2010effective programmes of prophylaxis targeted at the individual subject level.<\/jats:p><\/jats:sec>","DOI":"10.1111\/hae.14345","type":"journal-article","created":{"date-parts":[[2021,5,20]],"date-time":"2021-05-20T20:35:09Z","timestamp":1621542909000},"page":"666-673","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Patterns of joint damage in severe haemophilia A treated with prophylaxis"],"prefix":"10.1111","volume":"27","author":[{"given":"Rachel","family":"Goren","sequence":"first","affiliation":[{"name":"Institute of Health Policy, Management &amp; Evaluation The Dalla Lana School of Public Health University of Toronto Toronto ON Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eleanor","family":"Pullenayegum","sequence":"additional","affiliation":[{"name":"Institute of Health Policy, Management &amp; Evaluation The Dalla Lana School of Public Health University of Toronto Toronto ON Canada"},{"name":"Child Health Evaluative Sciences The Hospital for Sick Children Toronto ON Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Victor S.","family":"Blanchette","sequence":"additional","affiliation":[{"name":"Child Health Evaluative Sciences The Hospital for Sick Children Toronto ON Canada"},{"name":"Division of Hematology\/Oncology The Hospital for Sick Children Toronto ON Canada"},{"name":"Department of Pediatrics Faculty of Medicine University of Toronto Toronto ON Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5935-8101","authenticated-orcid":false,"given":"Saunya","family":"Dover","sequence":"additional","affiliation":[{"name":"Child Health Evaluative Sciences The Hospital for Sick Children Toronto ON Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5350-1763","authenticated-orcid":false,"given":"Manuel","family":"Carcao","sequence":"additional","affiliation":[{"name":"Child Health Evaluative Sciences The Hospital for Sick Children Toronto ON Canada"},{"name":"Division of Hematology\/Oncology The Hospital for Sick Children Toronto ON Canada"},{"name":"Department of Pediatrics Faculty of Medicine University of Toronto Toronto ON Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sara J.","family":"Israels","sequence":"additional","affiliation":[{"name":"Department of Pediatrics and Child Health University of Manitoba Winnipeg MB Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anthony","family":"Chan","sequence":"additional","affiliation":[{"name":"Department of Pediatrics McMaster Children\u2019s Hospital McMaster University Hamilton ON Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2861-9441","authenticated-orcid":false,"given":"Georges E.","family":"Rivard","sequence":"additional","affiliation":[{"name":"Division of Hematology\/Oncology Department of Pediatrics CHU Sainte\u2010Justine Montreal QC Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"MacGregor","family":"Steele","sequence":"additional","affiliation":[{"name":"Section of Pediatric Hematology Department of Pediatrics University of Calgary and Alberta Children\u2019s Hospital Calgary AB 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