{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,10]],"date-time":"2026-06-10T19:56:29Z","timestamp":1781121389937,"version":"3.54.1"},"reference-count":18,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2023,2,15]],"date-time":"2023-02-15T00:00:00Z","timestamp":1676419200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":["pericles.pericles-gcp.literatumonline.com"],"crossmark-restriction":true},"short-container-title":["Haemophilia"],"published-print":{"date-parts":[[2023,7]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Introduction<\/jats:title><jats:p>Good health\u2010related quality of life (HRQoL) is an important goal in the treatment of persons with haemophilia B (PwHB). Studies focusing on this population are limited, however, and data are insufficient.<\/jats:p><\/jats:sec><jats:sec><jats:title>Aim<\/jats:title><jats:p>To assess the HRQoL in PwHB and to compare this to data on persons with haemophilia A (PwHA), as well as to evaluate the impact of joint health on HRQoL and to identify areas of insufficient care.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>The B\u2010NORD study enrolled persons with severe haemophilia B and matched controls with haemophilia A. HRQoL was assessed using the EQ\u20105D\u20103L questionnaire and joint health using Haemophilia Joint Health Score 2.1 (HJHS).<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The EQ\u20105D\u20103L was completed by 63 PwHB and 63 PwHA. Mobility problems were reported by 46% of PwHB and 44% of PwHA, pain\/discomfort by 62% and 56%, and anxiety\/depression by 33% and 17%, respectively. No significant difference was observed between PwHA and PwHB in EQ\u20105D profiles, level sum score, EQ\u20105D index (PwHB mean .80, PwHA mean .83, <jats:italic>p<\/jats:italic>\u00a0=\u00a0.24), or EQ VAS score (PwHB: mean 70, PwHA: mean 77, <jats:italic>p<\/jats:italic>\u00a0=\u00a0.061). Linear regression adjusted for age demonstrated that an increase in HJHS score was associated with a significant decrease in both EQ\u20105D index (B \u2010.003, R<jats:sub>2<\/jats:sub> .22) and EQ VAS score (B \u2010.37, R<jats:sub>2<\/jats:sub> .17).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Despite the majority of patients being treated with prophylaxis, impaired HRQoL was reported in both PwHB and PwHA. No differences in HRQoL were found between the two groups. Impaired joint health had a significant negative impact on HRQoL.<\/jats:p><\/jats:sec>","DOI":"10.1111\/hae.14759","type":"journal-article","created":{"date-parts":[[2023,2,15]],"date-time":"2023-02-15T19:50:48Z","timestamp":1676490648000},"page":"987-996","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":11,"title":["No difference in quality of life between persons with severe haemophilia A and B"],"prefix":"10.1111","volume":"29","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2854-3280","authenticated-orcid":false,"given":"Kristina","family":"Kihlberg","sequence":"first","affiliation":[{"name":"Clinical Coagulation Research, Department of Translational Medicine Lund University  Malm\u00f6 Sweden"},{"name":"Department of Haematology, Oncology and Radiation Physics, Centre for Thrombosis and Haemostasis Sk\u00e5ne University Hospital  Malm\u00f6 Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fariba","family":"Baghaei","sequence":"additional","affiliation":[{"name":"Department of Haematology and Coagulation Sahlgrenska University Hospital  Gothenburg Sweden"},{"name":"Department of Internal Medicine, Institute of Medicine Sahlgrenska Academy University of Gothenburg  Gothenburg Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria","family":"Bruzelius","sequence":"additional","affiliation":[{"name":"Department of Haematology Karolinska University Hospital  Stockholm Sweden"},{"name":"Department of Medicine Solna Karolinska Institutet  Stockholm Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0314-5275","authenticated-orcid":false,"given":"Eva","family":"Funding","sequence":"additional","affiliation":[{"name":"Department of Hematology Rigshospitalet  Copenhagen Denmark"},{"name":"Institute of Clinical Medicine Copenhagen University  Copenhagen Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"P\u00e5l","family":"Andre Holme","sequence":"additional","affiliation":[{"name":"Department of Haematology Oslo University Hospital  Oslo Norway"},{"name":"Institute of Clinical Medicine University of Oslo  Oslo Norway"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1911-3094","authenticated-orcid":false,"given":"Riitta","family":"Lassila","sequence":"additional","affiliation":[{"name":"Department of Haematology, Coagulation Disorders Unit, Comprehensive Cancer Centre Helsinki University Hospital  Helsinki Finland"},{"name":"Research Programme in Systems Oncology, Faculty of Medicine University of Helsinki  Helsinki Finland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5134-7288","authenticated-orcid":false,"given":"Vuokko","family":"Nummi","sequence":"additional","affiliation":[{"name":"Department of Haematology, Coagulation Disorders Unit, Comprehensive Cancer Centre Helsinki University Hospital  Helsinki Finland"},{"name":"Research Programme in Systems Oncology, Faculty of Medicine University of Helsinki  Helsinki Finland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7854-0371","authenticated-orcid":false,"given":"Susanna","family":"Ranta","sequence":"additional","affiliation":[{"name":"Paediatric Coagulation, Astrid Lindgren Children's Hospital Karolinska University Hospital  Stockholm Sweden"},{"name":"Paediatric Oncology, Department of Women's and Children's Health Karolinska Institutet  Stockholm Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6058-8350","authenticated-orcid":false,"given":"Nadine","family":"Gretenkort Andersson","sequence":"additional","affiliation":[{"name":"Department of Haematology, Oncology and Radiation Physics, Centre for Thrombosis and Haemostasis Sk\u00e5ne University Hospital  Malm\u00f6 Sweden"},{"name":"Department of Clinical Sciences and Paediatrics Lund University  Lund Sweden"},{"name":"Department of Paediatric Haematology and Oncology Sk\u00e5ne University Hospital  Lund Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1337-7195","authenticated-orcid":false,"given":"Erik","family":"Berntorp","sequence":"additional","affiliation":[{"name":"Clinical Coagulation Research, Department of Translational Medicine Lund University  Malm\u00f6 Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8500-2483","authenticated-orcid":false,"given":"Jan","family":"Astermark","sequence":"additional","affiliation":[{"name":"Clinical Coagulation Research, Department of Translational Medicine Lund University  Malm\u00f6 Sweden"},{"name":"Department of Haematology, Oncology and Radiation Physics, Centre for Thrombosis and Haemostasis Sk\u00e5ne University Hospital  Malm\u00f6 Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2023,2,15]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1111\/hae.14299"},{"key":"e_1_2_9_3_1","first-page":"195","volume-title":"Epidemiology and demography in public health","author":"Hays R","year":"2010"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/ejh.13055"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/hae.14525"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1111\/hae.13960"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365\u20102516.2012.02863.x"},{"key":"e_1_2_9_8_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2012.02765.x"},{"key":"e_1_2_9_9_1","doi-asserted-by":"publisher","DOI":"10.1111\/hae.14571"},{"key":"e_1_2_9_10_1","doi-asserted-by":"publisher","DOI":"10.1111\/hae.13231"},{"key":"e_1_2_9_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.thromres.2022.06.015"},{"key":"e_1_2_9_12_1","unstructured":"EuroQol Research Foundation.EQ\u20105D\u20103L User Guide.2018. Available from:https:\/\/euroqol.org\/publications\/user\u2010guides"},{"key":"e_1_2_9_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/0168-8510(90)90421-9"},{"key":"e_1_2_9_14_1","doi-asserted-by":"publisher","DOI":"10.1016\/0168-8510(90)90421-9"},{"key":"e_1_2_9_15_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2006.01312.x"},{"key":"e_1_2_9_16_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2006.01312.x"},{"key":"e_1_2_9_17_1","doi-asserted-by":"publisher","DOI":"10.1111\/hae.14105"},{"key":"e_1_2_9_18_1","doi-asserted-by":"publisher","DOI":"10.1007\/978-94-007-7596-1_3"},{"key":"e_1_2_9_19_1","doi-asserted-by":"publisher","DOI":"10.1186\/s12955-018-1029-1"}],"container-title":["Haemophilia"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/hae.14759","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1111\/hae.14759","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/hae.14759","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,5]],"date-time":"2023-09-05T12:46:01Z","timestamp":1693917961000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/hae.14759"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,2,15]]},"references-count":18,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2023,7]]}},"alternative-id":["10.1111\/hae.14759"],"URL":"https:\/\/doi.org\/10.1111\/hae.14759","archive":["Portico"],"relation":{},"ISSN":["1351-8216","1365-2516"],"issn-type":[{"value":"1351-8216","type":"print"},{"value":"1365-2516","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,2,15]]},"assertion":[{"value":"2022-12-12","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-01-29","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-02-15","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}