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Marfan syndrome and related heritable thoracic aortic aneurysms and dissections

(2015) CURRENT PHARMACEUTICAL DESIGN. 21(28). p.4061-4075
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Abstract
In this overview we aim to address a number of recent insights and developments regarding clinical aspects, etiology, and treatment of Heritable Thoracic Aortic Disease (H-TAD). We will focus on monogenetic disorders related to aortic aneurysms. H-TADs are rare but they provide a unique basis for the study of underlying pathogenetic pathways in the complex disease process of aneurysm formation. The understanding of pathomechanisms may help us to identify medical treatment targets to improve prognosis. Among the monogenetic aneurysm disorders, Marfan syndrome is considered as a paradigm entity and many insights are derived from the study of clinical, genetic and animal models for Marfan syndrome. We will therefore first provide a detailed overview of the various aspects of Marfan syndrome after which we will give an overview of related H-TAD entities.
Keywords
aortic aneurysm, Marfan syndrome, connective tissue disorders, heritable thoracic aortic disorders, EHLERS-DANLOS-SYNDROME, ARTERIAL-TORTUOSITY-SYNDROME, SHPRINTZEN-GOLDBERG SYNDROME, LEFT-VENTRICULAR FUNCTION, MITRAL-VALVE DYSFUNCTION, BETA BINDING-PROTEIN, SMOOTH-MUSCLE-CELL, SYNDROME TYPE-IV, TGF-BETA, ROOT DILATION

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Citation

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Chicago
De Backer, Julie, Marjolijn Renard, Laurence Campens, Laura Muiño Mosquera, Anne De Paepe, Paul Coucke, Bert Callewaert, and Yskert von Kodolitsch. 2015. “Marfan Syndrome and Related Heritable Thoracic Aortic Aneurysms and Dissections.” Current Pharmaceutical Design 21 (28): 4061–4075.
APA
De Backer, Julie, Renard, M., Campens, L., Muiño Mosquera, L., De Paepe, A., Coucke, P., Callewaert, B., et al. (2015). Marfan syndrome and related heritable thoracic aortic aneurysms and dissections. CURRENT PHARMACEUTICAL DESIGN, 21(28), 4061–4075.
Vancouver
1.
De Backer J, Renard M, Campens L, Muiño Mosquera L, De Paepe A, Coucke P, et al. Marfan syndrome and related heritable thoracic aortic aneurysms and dissections. CURRENT PHARMACEUTICAL DESIGN. 2015;21(28):4061–75.
MLA
De Backer, Julie, Marjolijn Renard, Laurence Campens, et al. “Marfan Syndrome and Related Heritable Thoracic Aortic Aneurysms and Dissections.” CURRENT PHARMACEUTICAL DESIGN 21.28 (2015): 4061–4075. Print.
@article{6968149,
  abstract     = {In this overview we aim to address a number of recent insights and developments regarding clinical aspects, etiology, and treatment of Heritable Thoracic Aortic Disease (H-TAD). We will focus on monogenetic disorders related to aortic aneurysms. H-TADs are rare but they provide a unique basis for the study of underlying pathogenetic pathways in the complex disease process of aneurysm formation. The understanding of pathomechanisms may help us to identify medical treatment targets to improve prognosis. 
Among the monogenetic aneurysm disorders, Marfan syndrome is considered as a paradigm entity and many insights are derived from the study of clinical, genetic and animal models for Marfan syndrome. We will therefore first provide a detailed overview of the various aspects of Marfan syndrome after which we will give an overview of related H-TAD entities.},
  author       = {De Backer, Julie and Renard, Marjolijn and Campens, Laurence and Mui{\~n}o Mosquera, Laura and De Paepe, Anne and Coucke, Paul and Callewaert, Bert and von Kodolitsch, Yskert},
  issn         = {1381-6128},
  journal      = {CURRENT PHARMACEUTICAL DESIGN},
  keyword      = {aortic aneurysm,Marfan syndrome,connective tissue disorders,heritable thoracic aortic disorders,EHLERS-DANLOS-SYNDROME,ARTERIAL-TORTUOSITY-SYNDROME,SHPRINTZEN-GOLDBERG SYNDROME,LEFT-VENTRICULAR FUNCTION,MITRAL-VALVE DYSFUNCTION,BETA BINDING-PROTEIN,SMOOTH-MUSCLE-CELL,SYNDROME TYPE-IV,TGF-BETA,ROOT DILATION},
  language     = {eng},
  number       = {28},
  pages        = {4061--4075},
  title        = {Marfan syndrome and related heritable thoracic aortic aneurysms and dissections},
  url          = {http://dx.doi.org/10.2174/1381612821666150826093152},
  volume       = {21},
  year         = {2015},
}

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