Searched for: subject:"Marfan%5C+syndrome"
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document
Busnadiego, O. (author), Gorbenko del Blanco, D. (author), González-Santamaría, J. (author), Habashi, J.P. (author), Calderon, J.F. (author), Sandoval, P. (author), Bedja, D. (author), Guinea-Viniegra, J. (author), Lopez-Cabrera, M. (author), Rosell-Garcia, T. (author), Snabel, J.M. (author), Hanemaaijer, R. (author), Forteza, A. (author), Dietz, H.C. (author), Egea, G. (author), Rodriguez-Pascual, F. (author)
Patients with Marfan syndrome (MFS) are at high risk of life-threatening aortic dissections. The condition is caused by mutations in the gene encoding fibrillin-1, an essential component in the formation of elastic fibers. While experimental findings in animal models of the disease have shown the involvement of transforming growth factor-β (TGF...
article 2015
document
Lindeman, J.H.N. (author), Ashcroft, B.A. (author), Beenakker, J.-W.M. (author), van Es, M. (author), Koekkoek, N.B.R. (author), Prins, F.A. (author), Tielemans, J.F. (author), Abdul-Hussien, H. (author), Bank, R.A. (author), Oosterkamp, T.H. (author), TNO Kwaliteit van Leven (author)
An aneurysm of the aorta is a common pathology characterized by segmentalweakeningof the artery.Althoughit isgenerally accepted that the vessel-wall weakening is caused by an impaired collagen metabolism, a clear association has been demonstrated only for rare syndromes such as the vascular type Ehlers-Danlos syndrome. Here we show that vessel...
article 2010
document
Fredriks, A.M. (author), van Buuren, S. (author), van Heel, W.J.M. (author), Dijkman-Neerincx, R.H.M. (author), Verloove-Vanhorick, S.P. (author), Wit, J.M. (author), TNO Kwaliteit van Leven (author)
Aims: To obtain age references for sitting height (SH), leg length (LL), and SH/H ratio in the Netherlands; to evaluate how SH standard deviation score (SDS), LL SDS, SH/H SDS, and SH/LL SDS are related to height SDS; and to study the usefulness of height corrected SH/H cut-off lines to detect Marfan syndrome and hypochondroplasia. Methods:...
article 2005
document
Meijboom, L.J. (author), Westerhof, B.E. (author), Nollen, G.J. (author), Spaan, J.A.E. (author), de Mol, B.A.J.M. (author), Jacobs, M.J.H.M. (author), Mulder, B.J.M. (author), TNO Biomedical Instrumentation (author)
Objective: β-blocking therapy is the standard therapy in non-operated Marfan patients, however its efficacy after entire aortic replacement is unknown. The aim of this study was to describe the influence of (nearly) entire aortic replacement and β-blocking therapy on blood pressure and wave reflections in Marfan patients. Methods: Four Marfan...
article 2004
document
Nollen, G.J. (author), Westerhof, B.E. (author), Groenink, M. (author), Osnabrugge, A. (author), van der Wall, E.E. (author), Mulder, B.J.M. (author), TNO Industrie (author)
Objective: To investigate the heterogeneous response to β blockade in patients with Marfan syndrome by non-invasive assessment of the aortic pressure-area curve. Design and patients: 25 patients with the Marfan syndrome who used β blocking agents (mean (SD) age, 29 (10) years; 20 men, five women), seven without β blockade (34 (14) years; five...
article 2004
Searched for: subject:"Marfan%5C+syndrome"
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