{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,28]],"date-time":"2026-09-28T04:54:59Z","timestamp":1790571299350,"version":"4.1.0"},"reference-count":17,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2025,11,28]],"date-time":"2025-11-28T00:00:00Z","timestamp":1764288000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2025,11,28]],"date-time":"2025-11-28T00:00:00Z","timestamp":1764288000000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"funder":[{"DOI":"10.13039\/100008373","name":"Takeda Pharmaceutical Company","doi-asserted-by":"publisher","award":["IISR\u20102020\u2010103252"],"award-info":[{"award-number":["IISR\u20102020\u2010103252"]}],"id":[{"id":"10.13039\/100008373","id-type":"DOI","asserted-by":"publisher"}]}],"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>Background<\/jats:title>\n                    <jats:p>Modern prophylaxis in haemophilia aims not only to prevent bleeding but also to ensure a quality of life (QoL) comparable to healthy peers. Real\u2010world data on health\u2010related QoL (HRQoL) in children receiving personalised pharmacokinetic (PK)\u2010guided prophylaxis are limited.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Aim<\/jats:title>\n                    <jats:p>To compare HRQoL outcomes in Polish children with haemophilia A who switched from standard plasma\u2010derived factor VIII (pdFVIII) prophylaxis to recombinant factor VIII (rFVIII), either as standard or PK\u2010tailored prophylaxis.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>\n                      In this nationwide study, 131 boys with severe or moderate haemophilia A were assessed before and \u226526 weeks after switching from pdFVIII to rFVIII. Patients either continued standard prophylaxis (Group 1,\n                      <jats:italic>n<\/jats:italic>\n                      \u00a0=\u00a028) or started PK\u2010tailored prophylaxis (Group 2,\n                      <jats:italic>n<\/jats:italic>\n                      \u00a0=\u00a0103). Bleeding outcomes (annualised bleeding rate [ABR], annualised joint bleeding rate [AJBR]) and QoL (EQ\u20105D\u20105L, Visual Analogue Scale [VAS]) were compared using Student's\n                      <jats:italic>t<\/jats:italic>\n                      \u2010test and Wilcoxon test.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Switching to rFVIII significantly reduced ABR (1.73\u21920.76;\n                      <jats:italic>p<\/jats:italic>\n                      \u00a0=\u00a00.004) and AJBR (0.65\u21920.33;\n                      <jats:italic>p<\/jats:italic>\n                      \u00a0=\u00a00.009). QoL improved overall, with higher VAS scores (88.6\u219291.2;\n                      <jats:italic>p<\/jats:italic>\n                      \u00a0=\u00a00.02) and lower EQ\u20105D\u20105L scores (5.78\u21925.54;\n                      <jats:italic>p<\/jats:italic>\n                      \u00a0=\u00a00.01). Improvements were most pronounced in Group 2, where both bleeding rates and QoL significantly improved, despite a higher weekly number of i.v. injections. No significant QoL changes were observed in Group 1.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>Personalised PK\u2010based rFVIII prophylaxis leads to significant improvements in bleeding control and HRQoL in Polish children with haemophilia A. These results support the integration of PK\u2010guided prophylaxis into routine clinical practice.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/hae.70177","type":"journal-article","created":{"date-parts":[[2025,11,28]],"date-time":"2025-11-28T16:49:21Z","timestamp":1764348561000},"page":"98-105","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Real\u2010World Data on Quality of Life in Relation to the Type of Prophylaxis in Polish Children With Haemophilia A\u2014A Nationwide Study"],"prefix":"10.1111","volume":"32","author":[{"given":"Klaudia","family":"Pacz\u00f3ska","sequence":"first","affiliation":[{"name":"Department of Pediatrics and Hemato\u2010Oncology Pomeranian Medical University in Szczecin  Szczecin Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wanda","family":"Badowska","sequence":"additional","affiliation":[{"name":"Department of Pediatric Oncology and Hematology School of Medicine University of Warmia Mazury in Olsztyn  Olsztyn Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Halina","family":"Bobrowska","sequence":"additional","affiliation":[{"name":"Wielkopolska Child Health Center  Poznan Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"\u0141ucja","family":"Dakowicz","sequence":"additional","affiliation":[{"name":"Department of Pediatric Oncology and Hematology Medical University of Bialystok  Bia\u0142ystok Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Grzegorz","family":"Dobaczewski","sequence":"additional","affiliation":[{"name":"Department of Bone Marrow Transplantation Pediatric Oncology and Hematology, Wroclaw Medical University  Wroclaw Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"El\u017cbieta","family":"Latos\u2010Gra\u017cy\u0144ska","sequence":"additional","affiliation":[{"name":"Department of Bone Marrow Transplantation Pediatric Oncology and Hematology, Wroclaw Medical University  Wroclaw Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gra\u017cyna","family":"Karolczyk","sequence":"additional","affiliation":[{"name":"II Department of Pediatrics Collegium Medicum, Jan Kochanowski University  Kielce Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anna","family":"Klukowska","sequence":"additional","affiliation":[{"name":"Department of Pediatric Oncology Hematology and Transplantation, Medical University of Warsaw  Warsaw Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrzej","family":"Ko\u0142tan","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Hematology and Oncology, Collegium Medicum, Nicolaus Copernicus University  Bydgoszcz Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Magdalena","family":"Wojdalska","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Oncology and Hematology, Medical University of Lodz  Lodz Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pawe\u0142","family":"\u0141aguna","sequence":"additional","affiliation":[{"name":"Department of Pediatric Oncology Hematology and Transplantation, Medical University of Warsaw  Warsaw Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Danuta","family":"Pietrys","sequence":"additional","affiliation":[{"name":"Department of Pediatric Oncology and Hematology Collegium Medicum, Jagiellonian University  Krakow Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julia","family":"Rado\u0144\u2010Proskura","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Hematology and Oncology, Medical University of Gda\u0144sk  Gda\u0144sk Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Monika","family":"Radwa\u0144ska","sequence":"additional","affiliation":[{"name":"Department of Pediatric Oncology and Hematology College of Medical Sciences University of Rzeszow  Rzeszow Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Iwona","family":"Rura\u0144ska","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Hematology and Oncology, Medical University of Silesia  Katowice Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tomasz","family":"Szczepa\u0144ski","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Hematology and Oncology, Medical University of Silesia  Katowice Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dariusz","family":"Wasi\u0144ski","sequence":"additional","affiliation":[{"name":"Department of Pediatric Hematology and Oncology Collegium Medicum, University of Zielona Gora  Gora Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Irena","family":"Wo\u017anica\u2010Karczmarz","sequence":"additional","affiliation":[{"name":"Clinic of Pediatric Hematology and Oncology Medical University of Lublin  Lublin Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karolina","family":"Zielezi\u0144ska","sequence":"additional","affiliation":[{"name":"Department of Pediatrics and Hemato\u2010Oncology Pomeranian Medical University in Szczecin  Szczecin Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aleksandra","family":"Kr\u00f3lak","sequence":"additional","affiliation":[{"name":"Department of Pediatrics and Hemato\u2010Oncology Pomeranian Medical University in Szczecin  Szczecin Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Magdalena","family":"Bartnik","sequence":"additional","affiliation":[{"name":"Department of Pediatrics and Hemato\u2010Oncology Pomeranian Medical University in Szczecin  Szczecin Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hubert","family":"Kluk","sequence":"additional","affiliation":[{"name":"Department of Pediatrics and Hemato\u2010Oncology Pomeranian Medical University in Szczecin  Szczecin Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tomasz","family":"Urasi\u0144ski","sequence":"additional","affiliation":[{"name":"Department of Pediatrics and Hemato\u2010Oncology Pomeranian Medical University in Szczecin  Szczecin Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tomasz","family":"Ociepa","sequence":"additional","affiliation":[{"name":"Department of Pediatrics and Hemato\u2010Oncology Pomeranian Medical University in Szczecin  Szczecin Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2025,11,28]]},"reference":[{"key":"e_1_2_11_2_1","doi-asserted-by":"publisher","DOI":"10.1111\/hae.14046"},{"key":"e_1_2_11_3_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2648.1993.18010032.x"},{"issue":"3","key":"e_1_2_11_4_1","first-page":"e99","article-title":"A Brief History of 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J.","year":"2013","journal-title":"Hamostaseologie"},{"key":"e_1_2_11_16_1","doi-asserted-by":"publisher","DOI":"10.1111\/hae.13985"},{"key":"e_1_2_11_17_1","unstructured":"Canadian Hemophilia Society.Playing it Safe: Physical Activity and Exercise for People With Hemophilia https:\/\/www.hemophilia.ca\/files\/PlayingItSafe.pdf."},{"issue":"11","key":"e_1_2_11_18_1","first-page":"1064","article-title":"Sports and Children With Hemophilia: Current Trends","volume":"8","author":"Moretti L.","year":"2021","journal-title":"Children (Basel)"}],"container-title":["Haemophilia"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/hae.70177","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1111\/hae.70177","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/hae.70177","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,9,28]],"date-time":"2026-09-28T03:55:37Z","timestamp":1790567737000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/hae.70177"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,11,28]]},"references-count":17,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2026,1]]}},"alternative-id":["10.1111\/hae.70177"],"URL":"https:\/\/doi.org\/10.1111\/hae.70177","archive":["Portico"],"relation":{},"ISSN":["1351-8216","1365-2516"],"issn-type":[{"value":"1351-8216","type":"print"},{"value":"1365-2516","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,11,28]]},"assertion":[{"value":"2025-08-19","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-11-14","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-11-28","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}