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The aims of this follow\u2010up study were to explore how clinical features change over a 10\u2010year period in the same Norwegian MFS cohort. In 2003\u20132004, we investigated 105 adults for all manifestations in the 1996 Ghent nosology. Ten years later, we performed follow\u2010up investigations of the survivors (<jats:italic>n<\/jats:italic> = 48) who consented. Forty\u2010six fulfilled the revised Ghent criteria. Median age: females 51\u2009years, range 32\u201380\u2009years; males 45\u2009years, range 30\u201367\u2009years. New aortic root dilatation was detected in patients up to 70\u2009years. Ascending aortic pathology was diagnosed in 93 versus 72% at baseline. Sixty\u2010five percent had undergone aortic surgery compared to 39% at baseline. Pulmonary trunk mean diameter had increased significantly compared to baseline. From inclusion to follow\u2010up, two patients (three eyes) developed ectopia lentis, four developed dural ectasia, four developed scoliosis, three developed incisional or recurrent herniae, and 14 developed hindfoot deformity. No changes were found regarding protrusio acetabuli, spontaneous pneumothorax, or striae atrophicae. The study confirms that knowledge of incidence and progression of organ manifestations throughout life is important for diagnosis, treatment, and follow\u2010up of patients with verified or suspected MFS.<\/jats:p>","DOI":"10.1002\/ajmg.a.61441","type":"journal-article","created":{"date-parts":[[2019,12,11]],"date-time":"2019-12-11T11:55:53Z","timestamp":1576065353000},"page":"397-408","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":17,"title":["Marfan syndrome: Evolving organ manifestations\u2014A 10\u2010year follow\u2010up study"],"prefix":"10.1002","volume":"182","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2792-8864","authenticated-orcid":false,"given":"Thy Thy","family":"Vanem","sequence":"first","affiliation":[{"name":"Faculty of Medicine, University of Oslo Institute of Clinical Medicine  Oslo Norway"},{"name":"Department of Cardiothoracic Surgery Oslo University Hospital (OUH)  Oslo 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