{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,5]],"date-time":"2026-08-05T06:52:43Z","timestamp":1785912763237,"version":"3.56.0"},"reference-count":28,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2022,12,26]],"date-time":"2022-12-26T00:00:00Z","timestamp":1672012800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Haemophilia"],"published-print":{"date-parts":[[2023,3]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Introduction<\/jats:title><jats:p>The prophylactic regimen in children with severe haemophilia is suggested in various publications and guidelines. Few data exist on its implementation in clinical practice.<\/jats:p><\/jats:sec><jats:sec><jats:title>Aim<\/jats:title><jats:p>To investigate the implementation of primary prophylaxis based on real\u2010life data from PedNet during the last 20 years.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>All children from the PedNet cohort (<jats:italic>n<\/jats:italic>\u00a0=\u00a01260) with severe haemophilia A (SHA) or severe haemophilia B (SHB), FVIII\/IX\u00a0&lt;\u00a0.01 IU\/mL, born between 2000 and 2009 (Cohort I; SHA <jats:italic>n<\/jats:italic>\u00a0=\u00a0662; SHB <jats:italic>n<\/jats:italic>\u00a0=\u00a088) and 2010\u20132019 (Cohort II; SHA <jats:italic>n<\/jats:italic>\u00a0=\u00a0598; SHB <jats:italic>n<\/jats:italic>\u00a0=\u00a094) were included.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>In SHA, the median age at start of prophylaxis was 17.3 months (IQR; 12.5\u201326.1) in Cohort I which decreased to 13.1 months (IQR; 10.4\u201319.1) in Cohort II (<jats:italic>p<\/jats:italic>\u00a0&lt;\u00a0.000). \u201cOnce\u2010a\u2010week\u201d prophylaxis at start increased from 49% to 68% (SHA) and 38% to 70% (SHB). FVIII doses were reduced from median 43.5 (IQR; 34.6\u201349.0) to 30.9\u00a0IU\/kg (IQR; 26.3\u201346.3), while dosing with FIX did not change. After 2010 approximately 60% of the patients with SHA and SHB started prophylaxis before any joint bleed. The number of CVADs needed in both cohorts was around 30%. Incidences of inhibitors were unchanged: SHA (\u223c31%) and SHB (\u223c10%). Sporadic cases were diagnosed significantly later (median 8.3 months; IQR; 3.7\u201311.9) and they had more joint bleeds before start of prophylaxis.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Primary prophylaxis nowadays starts at an earlier age: before any joint bleed (60% of patients with SHA and SHB). Approximately 70% started on a once\u2010weekly schedule with significantly reduced doses in SHA but unchanged in SHB.<\/jats:p><\/jats:sec>","DOI":"10.1111\/hae.14729","type":"journal-article","created":{"date-parts":[[2022,12,26]],"date-time":"2022-12-26T18:28:42Z","timestamp":1672079322000},"page":"498-504","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":12,"title":["Primary prophylaxis in children with severe haemophilia A and B\u2014Implementation over the last 20 years as illustrated in real\u2010world data in the PedNet cohorts"],"prefix":"10.1111","volume":"29","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3999-8747","authenticated-orcid":false,"given":"Rolf","family":"Ljung","sequence":"first","affiliation":[{"name":"Department of Clinical Sciences, Department of Paediatrics, Lund University  Lund Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3521-1863","authenticated-orcid":false,"given":"Marloes","family":"de Kovel","sequence":"additional","affiliation":[{"name":"PedNet Haemophilia Research Foundation  Baarn the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2553-2324","authenticated-orcid":false,"given":"H. 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