{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,3,27]],"date-time":"2025-03-27T04:08:23Z","timestamp":1743048503530,"version":"3.40.3"},"reference-count":35,"publisher":"Cambridge University Press (CUP)","issue":"12","license":[{"start":{"date-parts":[[2024,10,2]],"date-time":"2024-10-02T00:00:00Z","timestamp":1727827200000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["cambridge.org"],"crossmark-restriction":true},"short-container-title":["Cardiol Young"],"published-print":{"date-parts":[[2024,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec id=\"S1047951124025770_as1\"><jats:title>Background and aim:<\/jats:title><jats:p>Marfan syndrome is a rare genetic connective tissue disorder. Research on health-related quality of life in Swedish patients is lacking. We aimed to examine health-related quality of life in patients with Marfan syndrome with respect to reference values, sex, and age.<\/jats:p><\/jats:sec><jats:sec id=\"S1047951124025770_as2\"><jats:title>Methods:<\/jats:title><jats:p>Using the registry for adult CHD, Sahlgrenska University Hospital\/\u00d6stra Hospital, between 1 April 2009 and 31 January 2023, we identified 1916 patients. Of these, we included 33 patients aged \u226518 years who were diagnosed with Marfan syndrome and had completed the 36-item Short-Form Health Survey.<\/jats:p><\/jats:sec><jats:sec id=\"S1047951124025770_as3\"><jats:title>Results:<\/jats:title><jats:p>The median age was 32 years (interquartile range 25.5\u201347.0) and 22 (66.7%) were men. Patients with Marfan syndrome had significantly lower values than reference values for all scales in the Short-Form Health Survey except bodily pain, role-emotional, and the physical component summary score. For both men and women with Marfan syndrome, vitality was the subscale with the greatest percentage difference in comparison with healthy reference values (82% in women and 73% in men). Furthermore, men reported significantly higher vitality levels than women (62.5 points, interquartile range 43.8\u201375.0 vs. 35 points, interquartile range 10.0\u201365.0, <jats:italic>p<\/jats:italic> = 0.026).<\/jats:p><\/jats:sec><jats:sec id=\"S1047951124025770_as4\"><jats:title>Conclusion:<\/jats:title><jats:p>Adults with Marfan syndrome in Sweden showed lower health-related quality of life levels in comparison with reference values for most Short-Form Health Survey scales, and there were differences between patients with Marfan syndrome in terms of sex and age.<\/jats:p><\/jats:sec>","DOI":"10.1017\/s1047951124025770","type":"journal-article","created":{"date-parts":[[2024,10,2]],"date-time":"2024-10-02T07:34:35Z","timestamp":1727854475000},"page":"2514-2520","update-policy":"https:\/\/doi.org\/10.1017\/policypage","source":"Crossref","is-referenced-by-count":0,"title":["Health-related quality of life in adults with Marfan syndrome"],"prefix":"10.1017","volume":"34","author":[{"given":"Anna","family":"Stanek S\u00f6rner","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maja","family":"Enelund","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"\u00c5sa","family":"Cider","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4148-4844","authenticated-orcid":false,"given":"Linda","family":"Ashman Kr\u00f6\u00f6nstr\u00f6m","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"56","published-online":{"date-parts":[[2024,10,2]]},"reference":[{"key":"S1047951124025770_ref15","unstructured":"15. World Health Organization. Constitution of the World Health Organization. 2006. https:\/\/www.who.int\/about\/accountability\/governance\/constitution. 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The National Board of Health and Welfare. The National Board of Health and Welfare. Marfan syndrome, 2023. https:\/\/www.socialstyrelsen.se\/kunskapsstod-och-regler\/omraden\/sallsynta-halsotillstand\/marfans-syndrom\/ [Accessed 4 Jan 2023]."},{"key":"S1047951124025770_ref27","doi-asserted-by":"publisher","DOI":"10.1186\/s12931-019-1010-5"},{"key":"S1047951124025770_ref25","doi-asserted-by":"publisher","DOI":"10.1023\/A:1012552211996"},{"volume-title":"SF-36 health survey: manual and interpretation guide","year":"1993","author":"Ware","key":"S1047951124025770_ref22"},{"key":"S1047951124025770_ref34","doi-asserted-by":"publisher","DOI":"10.1186\/s13023-022-02198-9"},{"key":"S1047951124025770_ref29","doi-asserted-by":"publisher","DOI":"10.1097\/CORR.0000000000001341"},{"key":"S1047951124025770_ref30","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2017.04.026"},{"key":"S1047951124025770_ref4","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199007193230303"},{"key":"S1047951124025770_ref14","unstructured":"14. World Health Organization. WHOQOL : Measuring Quality of Life. 1995. https:\/\/www.who.int\/healthinfo\/survey\/whoqol-qualityoflife\/en\/. Accessed 12 Jan 2023."},{"key":"S1047951124025770_ref10","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.a.36574"},{"key":"S1047951124025770_ref21","doi-asserted-by":"publisher","DOI":"10.1017\/S104795112000075X"},{"key":"S1047951124025770_ref31","doi-asserted-by":"publisher","DOI":"10.1136\/bmj.306.6890.1437"},{"key":"S1047951124025770_ref6","unstructured":"6. Physical Activity Guidelines. 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Published by Cambridge University Press","name":"copyright","label":"Copyright","group":{"name":"copyright_and_licensing","label":"Copyright and Licensing"}},{"value":"This is an Open Access article, distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivatives licence (https:\/\/creativecommons.org\/licenses\/by\/4.0\/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided that no alterations are made and the original article is properly cited. The written permission of Cambridge University Press must be obtained prior to any commercial use and\/or adaptation of the article.","name":"license","label":"License","group":{"name":"copyright_and_licensing","label":"Copyright and Licensing"}},{"value":"This content has been made available to all.","name":"free","label":"Free to read"}]}}