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• W3167962912 abstract "Abstract Background Marfan syndrome (MFS) is a connective tissue disorder characterized by mutations in the fibrillin-1 (FBN1) gene. The most important manifestation is progressive enlargement of the aorta, ultimately leading to aortic dissection or rupture. Present day pharmacological treatment is based on drugs that decrease repetitive stress on the aorta and improve elastic properties and transport function of the aorta. Unfortunately, this is not sufficient for most patients and surgical resection and reconstruction of the enlarged part of the aorta is still necessary. We have shown earlier that the food supplement Resveratrol inhibits aortic growth and promotes aortic repair in a murine MFS model. Resveratrol is a natural polyphenol commonly found in the skin of berries and in nuts. Here, we hypothesize that Resveratrol treatment will also be protective against aortic dilatation and functional deterioration in humans with MFS. Methods/Design The RESVcue Marfan study is an investigator-initiated, prospective, pre-post observational, multicenter trial. Four specialized academic centers in the Netherlands will recruit 100 patients with genetically confirmed MFS. Patients will use Resveratrol for one year, on top of their ongoing medicinal regime. The primary endpoints consist of the following two main parameters; 1) aortic aneurysm expansion rate and 2) improvement of aortic functional indices. All indices will be measured before and after treatment by echocardiography and (4D flow) MRI. The 4D flow MRI allows for analysis of blood flow patterns and quantification of stresses on the aortic wall, distensibility and pulse wave velocity. Blood and skin samples will be taken to assess the effect of Resveratrol on potential biomarkers associated with Resveratrol metabolism and changes in the primary endpoints. Three psychological tests ( The Well-being questionnaire (SF-12), the Hospital Depression and Anxiety Scales (HADS) questionnaire and the Checklist Individual Strength (CIS)) will be taken by the MFS patients to assess the effect of Resveratrol on their well-being. Discussion The RESVcue Marfan trial may provide sufficient data on beneficial effects of Resveratrol on aortic aneurysm expansion rate, aortic functional properties, and biomarkers in patients with MFS to justify a future randomized trial in a larger cohort for a longer study period. Trial registration NL66127.018.18, approved on 24 September 2018. https://www.toetsingonline.nl/ Resveratrol as potential aortic growth inhibitor in patients with Marfan syndrome." @default.
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• W3167962912 date "2021-06-07" @default.
• W3167962912 modified "2023-10-15" @default.
• W3167962912 title "The Effect of Resveratrol on Aortic Function in Patients with Marfan Syndrome – Rationale and Design of the Resvcue Marfan Trial." @default.
• W3167962912 doi "https://doi.org/10.21203/rs.3.rs-434876/v1" @default.
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