Marfan Syndrome and Related Heritable Thoracic Aortic Aneurysms and Dissections
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Marfan Syndrome and Related Heritable Thoracic Aortic Aneurysms and Dissections. / De Backer, Julie; Renard, Marjolijn; Campens, Laurence; Mosquera, Laura Muino; De Paepe, Anne; Coucke, Paul; Callewaert, Bert; Kodolitsch, Yskert von.
In: CURR PHARM DESIGN, Vol. 21, No. 28, 2015, p. 4061-4075.Research output: SCORING: Contribution to journal › SCORING: Review article › Research
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TY - JOUR
T1 - Marfan Syndrome and Related Heritable Thoracic Aortic Aneurysms and Dissections
AU - De Backer, Julie
AU - Renard, Marjolijn
AU - Campens, Laurence
AU - Mosquera, Laura Muino
AU - De Paepe, Anne
AU - Coucke, Paul
AU - Callewaert, Bert
AU - Kodolitsch, Yskert von
PY - 2015
Y1 - 2015
N2 - 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.
AB - 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.
KW - Aneurysm, Dissecting/genetics
KW - Animals
KW - Aortic Aneurysm, Thoracic/genetics
KW - Disease Models, Animal
KW - Humans
KW - Marfan Syndrome/complications
KW - Prognosis
U2 - 10.2174/1381612821666150826093152
DO - 10.2174/1381612821666150826093152
M3 - SCORING: Review article
C2 - 26306841
VL - 21
SP - 4061
EP - 4075
JO - CURR PHARM DESIGN
JF - CURR PHARM DESIGN
SN - 1381-6128
IS - 28
ER -