{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,14]],"date-time":"2026-10-14T07:00:25Z","timestamp":1791961225757,"version":"4.3.4"},"reference-count":61,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2025,2,14]],"date-time":"2025-02-14T00:00:00Z","timestamp":1739491200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2025,2,14]],"date-time":"2025-02-14T00:00:00Z","timestamp":1739491200000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Haemophilia"],"published-print":{"date-parts":[[2025,3]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Prophylaxis with coagulation factor concentrates is the mainstay of treatment in severe hemophilia A and B. Data on bleeding rates in persons with congenital haemophilia B (PwcHB) receiving prophylaxis are inconsistent.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Aim<\/jats:title>\n                    <jats:p>This systematic review and meta\u2010analysis were aimed at assessing bleeding outcomes, including annualised bleeding rates (ABR) and the proportion of patients with zero bleeding events, in PwcHB receiving prophylaxis with plasma\u2010derived or recombinant FIX products with standard (rSHL) or extended half\u2010life (rEHL).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>A systematic search was conducted using the bibliographic database Medline, Embase and Cochrane Central Register. The protocol was registered on PROSPERO (registration number: CRD42024592785).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      The search yielded 2440 citations and a total of 42 studies (2 randomised and 40 nonrandomised) were included in the final analysis. The pooled estimated mean (95% confidence interval [CI]) ABR was significantly lower in PwcHB treated prophylactically with rEHL FIX than in those receiving rSHL FIX products (1.29 [95% CI: 0.91, 1.66] vs. 3.12 [95% CI: 2.63, 3.62],\n                      <jats:italic>p<\/jats:italic>\n                      \u00a0&lt;\u00a00.01). The proportion of participants with zero bleeding events was significantly higher in PwcHB treated prophylactically with rEHL FIX than in those receiving rSHL FIX (0.53 [95% CI: 0.37, 0.69] vs. 0.24 [95% CI: 0.14, 0.39],\n                      <jats:italic>p<\/jats:italic>\n                      = 0.01). The ABR did not differ according to age groups (more or less than 12 years).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>The results of this meta\u2010analysis suggest that compared to standard half\u2010life FIX concentrates, prophylaxis with rEHL FIX products is associated with a reduction in ABR and a higher proportion of patients with no bleeding episodes.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/hae.70006","type":"journal-article","created":{"date-parts":[[2025,2,14]],"date-time":"2025-02-14T05:49:46Z","timestamp":1739512186000},"page":"173-186","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Bleeding Episodes in Patients With Haemophilia B Receiving Prophylactic Factor IX Treatment: A Systematic Review and Meta\u2010Analysis"],"prefix":"10.1111","volume":"31","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8795-0580","authenticated-orcid":false,"given":"Massimo","family":"Franchini","sequence":"first","affiliation":[{"name":"Department of Transfusion Medicine and Hematology Carlo Poma Hospital  Mantua Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0954-013X","authenticated-orcid":false,"given":"Samantha","family":"Pasca","sequence":"additional","affiliation":[{"name":"Transfusion and Immunohematology\u00a0Department Azienda Provinciale Servizi Sanitari (APSS) \u2010 Trento  Trento Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carlo","family":"Mengoli","sequence":"additional","affiliation":[{"name":"Department of Transfusion Medicine and Hematology Carlo Poma Hospital  Mantua Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniele","family":"Focosi","sequence":"additional","affiliation":[{"name":"North\u2010Western Tuscany Blood Bank Pisa University Hospital  Pisa Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1915-3897","authenticated-orcid":false,"given":"Pier Mannuccio","family":"Mannucci","sequence":"additional","affiliation":[{"name":"Fondazione IRCCS Ca' Granda\u2010Ospedale Maggiore Policlinico and University of Milan Angelo Bianchi Bonomi Hemophilia and Thrombosis Center  Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2025,2,14]]},"reference":[{"key":"e_1_2_8_2_1","volume-title":"Hemophilia B","author":"Alshaikli 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