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Master Thesis

Kystes rénaux simples et maladie aortique dans une population de patients atteints de maladie de Marfan et contrôles appariés

Abstract : Background: a link between simple renal cysts (SRC) and aortic aneurysm or dissection has been recently reported. The Marfan Syndrome (MFS) is associated with aortic diseases but SRC have never been studied in this population. Methods: all MFS patients with a mutation in the Fibrillin 1 gene who underwent a CT scan in the Reference Center for Marfan disease were included in the study. They were matched 1:1 for age and sex with controls without MFS. Results: between 2010 and 2016, 131 MFS patients and their matched controls were included. Mean age was 40±14 years with 42% of women. In MFS patients, SRC were associated with higher rates of aortic surgery (p=0.01) and arterial dissection (p=0.02). In multivariate analysis, 3 factors were associated with aortic surgery: male gender (p=0.02), higher aortic diameters (p<0.0001) and SRC (p<0.05). The proportion of SRC in MFS patients was 41% versus 21% in controls (p<0.0001). MFS patients had more cysts in all categories of age, the difference being more marked in young patients. The proportion of SRC was consistent with the severity of the arterial disease: 59% in dissections, 43% in aortic aneurysm surgery, 19% when no events and 21% in controls, p<0.009. Conclusion: this thesis reports for the first time an increased prevalence of SRC in MFS patients as compared with matched controls. SRC were associated with aortic events in this population and could be used as a marker of severity.
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Master Thesis
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Noémie Tencé. Kystes rénaux simples et maladie aortique dans une population de patients atteints de maladie de Marfan et contrôles appariés. Médecine humaine et pathologie. 2018. ⟨dumas-02304211⟩

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