Recent progress in understanding the natural and clinical histories of the Marfan syndrome
- PMID: 26908026
- DOI: 10.1016/j.tcm.2015.12.003
Recent progress in understanding the natural and clinical histories of the Marfan syndrome
Abstract
Over the past 4 decades, remarkable progress in understanding the cause, pathogenesis, and management of the MFS has led to an increase in life expectancy to near normal for most patients. Accompanying this increased life span has been the emergence of previously rare or unanticipated clinical problems. Despite much more detailed knowledge of the molecular, cellular, and tissue effects of a mutation in FBN1, targeted, effective therapy remains elusive. Until such precision medicine takes hold, management will depend on early diagnosis, regular scrutiny by imaging, chronic β-blockade, and perhaps ARBs, and prophylactic cardiothoracic surgery. Without question, MFS will remain a fertile subject for basic, translational, and clinical research for the foreseeable future.
Keywords: Aortic aneurysm; Fibrillin; Transforming growth factor-beta.
Copyright © 2016 Elsevier Inc. All rights reserved.
Comment in
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Editorial Commentary: Understanding Marfan syndrome, or "how not to invent the light bulb".Trends Cardiovasc Med. 2016 Jul;26(5):429-32. doi: 10.1016/j.tcm.2016.02.005. Epub 2016 Feb 23. Trends Cardiovasc Med. 2016. PMID: 27013137 Free PMC article. No abstract available.
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