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Ruptured abdominal aortic aneurysm repair in pediatric Marfan syndrome patient

Marfan syndrome is a well-described autosomal dominant connective tissue disorder with a constellation of anatomic characteristics including aortic degeneration as a result of the spontaneous mutation of the fibrillin gene, FBN1. Whereas life-threatening dissection and ascending aneurysmal rupture h...

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Autores principales: Lu, Joyce J., Slaikeu, Jason D., Wong, Peter Y.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5849780/
https://www.ncbi.nlm.nih.gov/pubmed/29541693
http://dx.doi.org/10.1016/j.jvscit.2017.11.008
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author Lu, Joyce J.
Slaikeu, Jason D.
Wong, Peter Y.
author_facet Lu, Joyce J.
Slaikeu, Jason D.
Wong, Peter Y.
author_sort Lu, Joyce J.
collection PubMed
description Marfan syndrome is a well-described autosomal dominant connective tissue disorder with a constellation of anatomic characteristics including aortic degeneration as a result of the spontaneous mutation of the fibrillin gene, FBN1. Whereas life-threatening dissection and ascending aneurysmal rupture have been thoroughly documented in the literature, aneurysms of the abdominal aorta and those present in the pediatric population have only rarely been reported. In this case report, we describe presentation, successful open surgical repair, and recovery of a pediatric Marfan syndrome patient with a ruptured abdominal aortic aneurysm.
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spelling pubmed-58497802018-03-14 Ruptured abdominal aortic aneurysm repair in pediatric Marfan syndrome patient Lu, Joyce J. Slaikeu, Jason D. Wong, Peter Y. J Vasc Surg Cases Innov Tech Aortic aneurysm and dissection Marfan syndrome is a well-described autosomal dominant connective tissue disorder with a constellation of anatomic characteristics including aortic degeneration as a result of the spontaneous mutation of the fibrillin gene, FBN1. Whereas life-threatening dissection and ascending aneurysmal rupture have been thoroughly documented in the literature, aneurysms of the abdominal aorta and those present in the pediatric population have only rarely been reported. In this case report, we describe presentation, successful open surgical repair, and recovery of a pediatric Marfan syndrome patient with a ruptured abdominal aortic aneurysm. Elsevier 2018-02-14 /pmc/articles/PMC5849780/ /pubmed/29541693 http://dx.doi.org/10.1016/j.jvscit.2017.11.008 Text en © 2018 The Authors http://creativecommons.org/licenses/by-nc-nd/4.0/ This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
spellingShingle Aortic aneurysm and dissection
Lu, Joyce J.
Slaikeu, Jason D.
Wong, Peter Y.
Ruptured abdominal aortic aneurysm repair in pediatric Marfan syndrome patient
title Ruptured abdominal aortic aneurysm repair in pediatric Marfan syndrome patient
title_full Ruptured abdominal aortic aneurysm repair in pediatric Marfan syndrome patient
title_fullStr Ruptured abdominal aortic aneurysm repair in pediatric Marfan syndrome patient
title_full_unstemmed Ruptured abdominal aortic aneurysm repair in pediatric Marfan syndrome patient
title_short Ruptured abdominal aortic aneurysm repair in pediatric Marfan syndrome patient
title_sort ruptured abdominal aortic aneurysm repair in pediatric marfan syndrome patient
topic Aortic aneurysm and dissection
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5849780/
https://www.ncbi.nlm.nih.gov/pubmed/29541693
http://dx.doi.org/10.1016/j.jvscit.2017.11.008
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AT wongpetery rupturedabdominalaorticaneurysmrepairinpediatricmarfansyndromepatient