Anna Stanek Sörner, Maja Enelund, Åsa Cider, Linda Ashman Kröönström
{"title":"马凡氏综合征成人患者的健康相关生活质量。","authors":"Anna Stanek Sörner, Maja Enelund, Åsa Cider, Linda Ashman Kröönström","doi":"10.1017/S1047951124025770","DOIUrl":null,"url":null,"abstract":"<p><strong>Background and aim: </strong>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.</p><p><strong>Methods: </strong>Using the registry for adult CHD, Sahlgrenska University Hospital/Östra Hospital, between 1 April 2009 and 31 January 2023, we identified 1916 patients. Of these, we included 33 patients aged ≥18 years who were diagnosed with Marfan syndrome and had completed the 36-item Short-Form Health Survey.</p><p><strong>Results: </strong>The median age was 32 years (interquartile range 25.5-47.0) 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 43.8-75.0 vs. 35 points, interquartile range 10.0-65.0, <i>p</i> = 0.026).</p><p><strong>Conclusion: </strong>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.</p>","PeriodicalId":9435,"journal":{"name":"Cardiology in the Young","volume":null,"pages":null},"PeriodicalIF":0.9000,"publicationDate":"2024-10-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Health-related quality of life in adults with Marfan syndrome.\",\"authors\":\"Anna Stanek Sörner, Maja Enelund, Åsa Cider, Linda Ashman Kröönström\",\"doi\":\"10.1017/S1047951124025770\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Background and aim: </strong>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.</p><p><strong>Methods: </strong>Using the registry for adult CHD, Sahlgrenska University Hospital/Östra Hospital, between 1 April 2009 and 31 January 2023, we identified 1916 patients. Of these, we included 33 patients aged ≥18 years who were diagnosed with Marfan syndrome and had completed the 36-item Short-Form Health Survey.</p><p><strong>Results: </strong>The median age was 32 years (interquartile range 25.5-47.0) 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 43.8-75.0 vs. 35 points, interquartile range 10.0-65.0, <i>p</i> = 0.026).</p><p><strong>Conclusion: </strong>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.</p>\",\"PeriodicalId\":9435,\"journal\":{\"name\":\"Cardiology in the Young\",\"volume\":null,\"pages\":null},\"PeriodicalIF\":0.9000,\"publicationDate\":\"2024-10-02\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Cardiology in the Young\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://doi.org/10.1017/S1047951124025770\",\"RegionNum\":4,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q4\",\"JCRName\":\"CARDIAC & CARDIOVASCULAR SYSTEMS\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Cardiology in the Young","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1017/S1047951124025770","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q4","JCRName":"CARDIAC & CARDIOVASCULAR SYSTEMS","Score":null,"Total":0}
Health-related quality of life in adults with Marfan syndrome.
Background and aim: 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.
Methods: Using the registry for adult CHD, Sahlgrenska University Hospital/Östra Hospital, between 1 April 2009 and 31 January 2023, we identified 1916 patients. Of these, we included 33 patients aged ≥18 years who were diagnosed with Marfan syndrome and had completed the 36-item Short-Form Health Survey.
Results: The median age was 32 years (interquartile range 25.5-47.0) 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 43.8-75.0 vs. 35 points, interquartile range 10.0-65.0, p = 0.026).
Conclusion: 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.
期刊介绍:
Cardiology in the Young is devoted to cardiovascular issues affecting the young, and the older patient suffering the sequels of congenital heart disease, or other cardiac diseases acquired in childhood. The journal serves the interests of all professionals concerned with these topics. By design, the journal is international and multidisciplinary in its approach, and members of the editorial board take an active role in the its mission, helping to make it the essential journal in paediatric cardiology. All aspects of paediatric cardiology are covered within the journal. The content includes original articles, brief reports, editorials, reviews, and papers devoted to continuing professional development.