{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,27]],"date-time":"2026-08-27T21:47:36Z","timestamp":1787867256029,"version":"build-2784847793"},"reference-count":43,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2016,9,9]],"date-time":"2016-09-09T00:00:00Z","timestamp":1473379200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Haemophilia"],"published-print":{"date-parts":[[2017,1]]},"abstract":"<jats:sec><jats:title>Introduction<\/jats:title><jats:p>von Willebrand disease (<jats:styled-content style=\"fixed-case\">VWD<\/jats:styled-content>) is the most common inherited bleeding disorder known in humans. Currently, studies investigating the health\u2010related quality of life (<jats:styled-content style=\"fixed-case\">HR<\/jats:styled-content>\u2010QoL) in <jats:styled-content style=\"fixed-case\">VWD<\/jats:styled-content> using standardized tools are limited, particularly among patients with mild decreases in von Willebrand factor or activity.<\/jats:p><\/jats:sec><jats:sec><jats:title>Aim<\/jats:title><jats:p>To determine <jats:styled-content style=\"fixed-case\">HR<\/jats:styled-content>\u2010QoL and its predictors among patients with mild, moderate and severe forms of <jats:styled-content style=\"fixed-case\">VWD<\/jats:styled-content>.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Patients with clinical diagnosis of <jats:styled-content style=\"fixed-case\">VWD<\/jats:styled-content> were recruited from a tertiary Inherited Bleeding Disorder Clinic. Upon informed consent, bleeding scores were obtained via a standardized, self\u2010administered tool. Each participant also completed a <jats:styled-content style=\"fixed-case\">HR<\/jats:styled-content>\u2010QoL questionnaire (<jats:styled-content style=\"fixed-case\">SF<\/jats:styled-content>\u201036). Analyses included paired <jats:italic>t<\/jats:italic>\u2010test, independent <jats:italic>t<\/jats:italic>\u2010test, one\u2010way <jats:sc>anova<\/jats:sc> and multivariate regression.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>A total of 102 patients were recruited (consent rate = 95%). Participants were 38 years on average (SD 14.8), 78% were female and 80% were diagnosed with <jats:styled-content style=\"fixed-case\">VWD<\/jats:styled-content> Type 1. Compared to age\u2010 and sex\u2010matched normative data, <jats:styled-content style=\"fixed-case\">VWD<\/jats:styled-content> patients had clinically and statistically significant reductions in seven of eight <jats:styled-content style=\"fixed-case\">HR<\/jats:styled-content>\u2010QoL domains and the physical and mental component summaries. Adjusted for age, sex, socioeconomic status and rurality, there was a trend towards lower physical component summary with increasing bleeding score, and lower mental domains with iron deficiency status (<jats:italic>P<\/jats:italic> = 0.07 and <jats:italic>P<\/jats:italic> = 0.08 respectively).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>This study is the first to examine the impact of <jats:styled-content style=\"fixed-case\">VWD<\/jats:styled-content> on <jats:styled-content style=\"fixed-case\">HR<\/jats:styled-content>\u2010QoL across disease severity while incorporating socioeconomic status and rurality. Significant reductions in <jats:styled-content style=\"fixed-case\">HR<\/jats:styled-content>\u2010QoL among <jats:styled-content style=\"fixed-case\">VWD<\/jats:styled-content> patients, especially the relationship between iron status and mental <jats:styled-content style=\"fixed-case\">HR<\/jats:styled-content>\u2010QoL, strengthen the rationale for prospective studies to evaluate the efficacy of iron replacement in this setting.<\/jats:p><\/jats:sec>","DOI":"10.1111\/hae.13062","type":"journal-article","created":{"date-parts":[[2016,9,9]],"date-time":"2016-09-09T16:23:47Z","timestamp":1473438227000},"page":"115-121","source":"Crossref","is-referenced-by-count":43,"title":["Relative contributions of bleeding scores and iron status on health\u2010related quality of life in von Willebrand disease: a cross\u2010sectional study"],"prefix":"10.1111","volume":"23","author":[{"given":"Y.","family":"Xu","sequence":"first","affiliation":[{"name":"School of Medicine Queen's University Kingston Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M.","family":"Deforest","sequence":"additional","affiliation":[{"name":"NCIC Clinical Trials Group Queen's University Kingston Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J.","family":"Grabell","sequence":"additional","affiliation":[{"name":"Department of Pathology and Molecular Medicine Queen's University Kingston Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"W.","family":"Hopman","sequence":"additional","affiliation":[{"name":"Clinical Research Centre, Kingston General Hospital Queen's University Kingston Canada"},{"name":"Department of Public Health Sciences Queen's University Kingston Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"P.","family":"James","sequence":"additional","affiliation":[{"name":"Department of Pathology and Molecular Medicine Queen's University Kingston Canada"},{"name":"Department of Medicine Queen's University Kingston Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2016,9,9]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2009.03661.x"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.pcl.2008.01.008"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.10327"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2006.01847.x"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2008.03182.x"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1097\/MLR.0b013e31829b1d84"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1377\/hlthaff.2010.0660"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2010.03864.x"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2001.00476.x"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.1111\/hae.12014"},{"key":"e_1_2_7_12_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00038-009-8057-x"},{"key":"e_1_2_7_13_1","doi-asserted-by":"publisher","DOI":"10.2105\/AJPH.87.9.1484"},{"key":"e_1_2_7_14_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1748-0361.2010.00277.x"},{"key":"e_1_2_7_15_1","doi-asserted-by":"publisher","DOI":"10.1136\/jech.2010.115378"},{"key":"e_1_2_7_16_1","doi-asserted-by":"publisher","DOI":"10.1155\/2015\/582163"},{"key":"e_1_2_7_17_1","doi-asserted-by":"publisher","DOI":"10.1093\/eurjhf\/hft083"},{"key":"e_1_2_7_18_1","unstructured":"World Health Organization.Serum ferritin concentrations for the assessment of iron status and iron deficiency in populations. 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