Part of Springer Nature

Artery Research

Volume 3, Issue 1, February 2009, Pages 9 - 16

Marfan and Marfan-like syndromes

Authors
J. De Backera, b, *, B. Loeysa, A. De Paepea
aDepartment of Medical Genetics, University Hospital Ghent, De Pintelaan 185, 9000 Ghent, Belgium
bDepartment of Cardiology, University Hospital Ghent, De Pintelaan 185, 9000 Ghent, Belgium
*Corresponding author at: Department of cardiology, University Hospital Ghent, De Pintelaan 185, 9000 Ghent, Belgium. Tel.: +32 9 332 56 27; fax: +32 9 332 49 70. E-mail address: julie.debacker@ugent.be (J. De Backer).
Corresponding Author
J. De Backer
Received 23 October 2008, Revised 8 January 2009, Accepted 19 January 2009, Available Online 3 March 2009.
DOI
https://doi.org/10.1016/j.artres.2009.01.003How to use a DOI?
Keywords
Marfan syndrome, Familial thoracic aortic aneurysm, genetics, Marfan-like syndrome
Abstract

With the help of both clinical and genetic diagnostic tools, the spectrum of inherited disorders affecting the arterial system has extended tremendously over the past decades. Discriminating these different entities is important since prognosis and treatment may differ substantially according to the diagnosis. Here we provide an overview of the current clinical and genetic knowledge on classic Marfan syndrome as well as on Marfan related disorders. Through our increased understanding of the pathophysiological mechanisms underlying aneurysm formation in these monogenetic conditions, new therapeutic strategies have emerged and are now being developed. This may serve as a nice example of translational medicine were detailed knowledge of the complex molecular pathways in rare disorders may help us to improve diagnosis and treatment of more common conditions.

Copyright
© 2009 Association for Research into Arterial Structure and Physiology. Published by Elsevier B.V. All rights reserved.
Open Access
This is an open access article distributed under the CC BY-NC license.

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Journal
Artery Research
Volume-Issue
3 - 1
Pages
9 - 16
Publication Date
2009/03
ISSN (Online)
1876-4401
ISSN (Print)
1872-9312
DOI
https://doi.org/10.1016/j.artres.2009.01.003How to use a DOI?
Copyright
© 2009 Association for Research into Arterial Structure and Physiology. Published by Elsevier B.V. All rights reserved.
Open Access
This is an open access article distributed under the CC BY-NC license.

Cite this article

TY  - JOUR
AU  - J. De Backer
AU  - B. Loeys
AU  - A. De Paepe
PY  - 2009
DA  - 2009/03
TI  - Marfan and Marfan-like syndromes
JO  - Artery Research
SP  - 9
EP  - 16
VL  - 3
IS  - 1
SN  - 1876-4401
UR  - https://doi.org/10.1016/j.artres.2009.01.003
DO  - https://doi.org/10.1016/j.artres.2009.01.003
ID  - DeBacker2009
ER  -