Artery Research

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Volume 15, Issue C, September 2016, Pages 21 - 29

Turner syndrome and guidelines for management of thoracic aortic disease: Appropriateness and utility

Authors
Hisham M.F Sherif*
Department of Cardiac Surgery, Christiana Hospital, Christiana Care Health System, Newark, DE, USA
*Corresponding author. Department of Cardiac Surgery, Christiana Hospital, Christiana Care Health System, 4755 Ogletown-Stanton Road, Suite 1E50, Newark, DE 19718, USA. Fax: +1 302 733 1986. E-mail address: hsherif@christianacare.org
Corresponding Author
Hisham M.F Sherif
Received 5 December 2014, Revised 23 February 2016, Accepted 18 July 2016, Available Online 4 August 2016.
DOI
10.1016/j.artres.2016.07.001How to use a DOI?
Keywords
Turner syndrome; Aortic aneurysm; Practice guidelines; Evidence-based practice
Abstract

Introduction: Current guidelines for management of thoracic aortic disease base clinical decision-making primarily on the aortic size. The risk of serious complications in genetically-triggered aortic disease is influenced by many other factors, currently not taken into account by the guidelines.

Methods: An extensive review of the medical literature using PubMed and Medline.

Results: Current guidelines do not make a distinction between atherosclerotic and nonatherosclerotic aneurysmal disease. Current guidelines for genetically triggered thoracic aortic disease are based on data from Marfan syndrome population. There remain significant differences between Turner syndrome and Marfan syndrome (in patient gender, fertility, stature, female sex hormone and growth hormone therapy as well as presence of coarctation) may have influence on the natural history of the disease and risk assessment.

Conclusions: Turner syndrome patients have a distinct set of factors influencing the development and natural history of their aortic disease, as opposed to Marfan syndrome patients. Therefore, a different set of clinical, genetic and biomarker parameters need to be devised for this condition. Recommendations from the current guidelines need further updates to better address indications for surveillance and therapeutic interventions in patients with Turner syndrome.

Copyright
© 2016 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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<Previous Article In Issue
Next Article In Issue>
Journal
Artery Research
Volume-Issue
15 - C
Pages
21 - 29
Publication Date
2016/08/04
ISSN (Online)
1876-4401
ISSN (Print)
1872-9312
DOI
10.1016/j.artres.2016.07.001How to use a DOI?
Copyright
© 2016 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

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AU  - Hisham M.F Sherif
PY  - 2016
DA  - 2016/08/04
TI  - Turner syndrome and guidelines for management of thoracic aortic disease: Appropriateness and utility
JO  - Artery Research
SP  - 21
EP  - 29
VL  - 15
IS  - C
SN  - 1876-4401
UR  - https://doi.org/10.1016/j.artres.2016.07.001
DO  - 10.1016/j.artres.2016.07.001
ID  - Sherif2016
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