{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,28]],"date-time":"2026-09-28T04:55:01Z","timestamp":1790571301412,"version":"4.1.0"},"reference-count":40,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2025,10,25]],"date-time":"2025-10-25T00:00:00Z","timestamp":1761350400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2025,10,25]],"date-time":"2025-10-25T00:00:00Z","timestamp":1761350400000},"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":[[2026,1]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Introduction<\/jats:title>\n                    <jats:p>Haemophilia B is an X\u2010linked recessive bleeding disorder caused by coagulation factor IX (FIX) deficiency. Treatment involves intravenous replacement of FIX. Recently, extended half\u2010life (EHL) FIX products have been introduced alongside standard half\u2010life (SHL) products to optimize therapy.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Aim<\/jats:title>\n                    <jats:p>This study evaluated bleeding rates, joint health, factor consumption, dosage, and health\u2010related quality of life (HRQoL) in patients switching from SHL\u2010 to EHL\u2010FIX products, as well as in those exclusively treated with EHL\u2010FIX.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Retrospective data from the medical records of 37 children with haemophilia B treated between 2010 and 2023 at two German Haemophilia Care Centres were analysed. HRQoL was assessed cross\u2010sectionally using haemophilia\u2010specific and generic questionnaires.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Twenty\u2010seven patients (median age: 12 years, range 2\u201319 years) switched from SHL\u2010 to EHL\u2010FIX, while 10 received EHL\u2010FIX from the start of prophylaxis. The mean annual bleeding rate (ABR) improved from 6.01\u00a0\u00b1\u00a07.01 (SHL) to 2.85\u00a0\u00b1\u00a03.42 (EHL). Factor consumption (159,577.8\u00a0\u00b1\u00a099,817.9\u00a0IU\/year), dosage (118.9\u00a0\u00b1\u00a050.3\u00a0IU\/kg\/week) and infusion frequency (145\u00a0\u00b1\u00a035.6 infusions\/year) decreased after switching (100,247.7\u00a0\u00b1\u00a046,268.6 IU\/year; 56.4\u00a0\u00b1\u00a023.7\u00a0IU\/kg\/week; 55.1\u00a0\u00b1\u00a09.8 infusions\/year). HRQoL improved in both self\u2010reports and parent reports. No severe adverse events occurred.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>Switching from SHL\u2010FIX to EHL\u2010FIX in children with haemophilia B is safe and may improve outcomes by reducing bleeding rates, infusion frequency, and factor consumption while enhancing joint health and HRQoL.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/hae.70157","type":"journal-article","created":{"date-parts":[[2025,10,25]],"date-time":"2025-10-25T11:25:53Z","timestamp":1761391553000},"page":"55-62","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["HAEMFIX: Impact of Switching From SHL\u2010FIX to EHL\u2010FIX in Patients With Haemophilia B"],"prefix":"10.1111","volume":"32","author":[{"given":"Jasmin","family":"Lonardi","sequence":"first","affiliation":[{"name":"Paediatric Thrombosis and Haemostasis Unit Paediatric Haemophilia Centre Dr. Von Hauner Children\u00b4s Hospital LMU Clinic  Munich Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Susan","family":"Halimeh","sequence":"additional","affiliation":[{"name":"Coagulation and Thrombosis Centre (GZRR)  Duisburg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sylvia","family":"von Mackensen","sequence":"additional","affiliation":[{"name":"Department of Medical Psychology University Medical Centre Hamburg\u2010Eppendorf  Hamburg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lisa","family":"Kleinlein","sequence":"additional","affiliation":[{"name":"Statistical Consulting Unit StaBLab LMU Munich  Munich Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Juliet","family":"Fleischer","sequence":"additional","affiliation":[{"name":"Statistical Consulting Unit StaBLab LMU Munich  Munich Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Henri","family":"Funk","sequence":"additional","affiliation":[{"name":"Statistical Consulting Unit StaBLab Department of Geography LMU Munich Munich Centre for Machine Learning (MCML)  Munich Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julia","family":"H\u00f6lz","sequence":"additional","affiliation":[{"name":"Paediatric Thrombosis and Haemostasis Unit Paediatric Haemophilia Centre Dr. Von Hauner Children\u00b4s Hospital LMU Clinic  Munich Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Johannes","family":"Holzapfel","sequence":"additional","affiliation":[{"name":"Paediatric Thrombosis and Haemostasis Unit Paediatric Haemophilia Centre Dr. Von Hauner Children\u00b4s Hospital LMU Clinic  Munich Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sabrina","family":"Juranek","sequence":"additional","affiliation":[{"name":"Paediatric Thrombosis 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