{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,8]],"date-time":"2026-10-08T21:23:39Z","timestamp":1791494619133,"version":"4.3.4"},"reference-count":35,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2016,2,8]],"date-time":"2016-02-08T00:00:00Z","timestamp":1454889600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100004191","name":"Novo Nordisk","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100004191","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Haemophilia"],"published-print":{"date-parts":[[2016,3]]},"abstract":"<jats:sec><jats:title>Introduction<\/jats:title><jats:p>Routine outcome assessment of prophylaxis should use validated tools, while balancing comprehensiveness and burden. Collecting overlapping information should be avoided.<\/jats:p><\/jats:sec><jats:sec><jats:title>Aim<\/jats:title><jats:p>To assess correlations between different outcome assessment tools in haemophilia.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>From an international cross\u2010sectional study, data on objective outcome (Haemophilia Joint Health Score (<jats:styled-content style=\"fixed-case\">HJHS<\/jats:styled-content> 2.1, range 0\u2013124), radiological Pettersson score) and self\u2010reported joint bleeding, Haemophilia Activities List (<jats:styled-content style=\"fixed-case\">HAL<\/jats:styled-content>, range 100\u20130), health\u2010related quality of life (<jats:styled-content style=\"fixed-case\">SF<\/jats:styled-content>\u201036, including five physical and five mental domain scores, range 100\u20130), and Utility (<jats:styled-content style=\"fixed-case\">SF<\/jats:styled-content>6D and <jats:styled-content style=\"fixed-case\">EQ<\/jats:styled-content>\u20105D, range 1.0\u20130) were extracted. Spearman's correlations were calculated: \u22650.8 very strong, 0.60\u20130.79 strong, 0.40\u20130.59 moderate.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Ninety patients with severe haemophilia, on prophylaxis since median age 3.4 years, were evaluated at median 25.5 years (range 16.0\u201337.6). Objective outcome was favourable (median <jats:styled-content style=\"fixed-case\">HJHS<\/jats:styled-content> 2.1 6 points, Pettersson score 9 points). Self\u2010reported outcome showed a median of 7 joint bleeds in 5 years, median <jats:styled-content style=\"fixed-case\">HAL<\/jats:styled-content> sum 96 points, high scores for physical domains of <jats:styled-content style=\"fixed-case\">SF<\/jats:styled-content>\u201036 (median 80\u201395) and high Utility values (median <jats:styled-content style=\"fixed-case\">SF<\/jats:styled-content>6D 0.87; <jats:styled-content style=\"fixed-case\">EQ<\/jats:styled-content>\u20105D 0.84). Physical examination (<jats:styled-content style=\"fixed-case\">HJHS<\/jats:styled-content> 2.1) showed strong correlation with radiological scores, moderate correlation with physical domains of the <jats:styled-content style=\"fixed-case\">SF<\/jats:styled-content>\u201036 and Utility, but no correlation with self\u2010reported bleeding or limitations in activities (<jats:styled-content style=\"fixed-case\">HAL<\/jats:styled-content>). Bleeding was not associated with any other outcome parameter. The <jats:styled-content style=\"fixed-case\">HAL<\/jats:styled-content> was only correlated with the <jats:styled-content style=\"fixed-case\">SF<\/jats:styled-content>36 \u2018Physical functioning\u2019 domain.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>For the evaluation of patients on early prophylaxis, information on bleeding should be complemented by objective joint assessment as well as self\u2010reported limitations in activities and quality of life.<\/jats:p><\/jats:sec>","DOI":"10.1111\/hae.12901","type":"journal-article","created":{"date-parts":[[2016,2,9]],"date-time":"2016-02-09T08:48:13Z","timestamp":1455007693000},"source":"Crossref","is-referenced-by-count":33,"title":["Evaluating outcome of prophylaxis in haemophilia: objective and self\u2010reported instruments should be combined"],"prefix":"10.1111","volume":"22","author":[{"given":"K.","family":"Fischer","sequence":"first","affiliation":[{"name":"Van Creveldkliniek Department of Hematology University Medical Center Utrecht Utrecht the Netherlands"},{"name":"Julius Center for Health Sciences and Primary Care University Medical Center Utrecht Utrecht the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A.","family":"Nijdam","sequence":"additional","affiliation":[{"name":"Van Creveldkliniek Department of Hematology University Medical Center Utrecht Utrecht the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M.","family":"Holmstr\u00f6m","sequence":"additional","affiliation":[{"name":"Coagulation Unit Karolinska University Hospital Stockholm Sweden"},{"name":"Department of Medicine Karolinska Institute Stockholm Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"P.","family":"Petrini","sequence":"additional","affiliation":[{"name":"Coagulation Unit Karolinska University Hospital Stockholm Sweden"},{"name":"Department of Paediatrics Karolinska University Hospital Stockholm Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"R.","family":"Ljung","sequence":"additional","affiliation":[{"name":"Department of Paediatrics Lund University Malm\u00f6 Sweden"},{"name":"Malm\u00f6 centre for Thrombosis and Haemostasis Sk\u00e5ne University Hospital Malm\u00f6 Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Y. T.","family":"van der Schouw","sequence":"additional","affiliation":[{"name":"Julius Center for Health Sciences and Primary Care University Medical Center Utrecht Utrecht the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"E.","family":"Berntorp","sequence":"additional","affiliation":[{"name":"Malm\u00f6 centre for Thrombosis and Haemostasis Sk\u00e5ne University Hospital Malm\u00f6 Sweden"},{"name":"Department of Clinical Sciences Lund University Sk\u00e5ne University Hospital Malm\u00f6 Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2016,2,8]]},"reference":[{"key":"e_1_2_8_2_1","first-page":"5","article-title":"Haemophilia in Sweden VII. 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