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Comprehensive review of aortic aneurysms, dissections, and cardiovascular complications in connective tissue disorders

Connective tissue disorders, including Marfan syndrome (MS) and Ehlers-Danlos syndrome (EDS), are characterized by genetic mutations affecting connective tissue structural integrity. These disorders significantly elevate the risk of aortic dissection, a life-threatening condition. This comprehensive...

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Autores principales: Elendu, Chukwuka, Amaechi, Dependable C., Elendu, Tochi C., Ibhiedu, Jennifer O., Torubiri, Augustina O., Okoye, Osinachi K.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Lippincott Williams & Wilkins 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10695548/
http://dx.doi.org/10.1097/MD.0000000000036499
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author Elendu, Chukwuka
Amaechi, Dependable C.
Elendu, Tochi C.
Ibhiedu, Jennifer O.
Torubiri, Augustina O.
Okoye, Osinachi K.
author_facet Elendu, Chukwuka
Amaechi, Dependable C.
Elendu, Tochi C.
Ibhiedu, Jennifer O.
Torubiri, Augustina O.
Okoye, Osinachi K.
author_sort Elendu, Chukwuka
collection PubMed
description Connective tissue disorders, including Marfan syndrome (MS) and Ehlers-Danlos syndrome (EDS), are characterized by genetic mutations affecting connective tissue structural integrity. These disorders significantly elevate the risk of aortic dissection, a life-threatening condition. This comprehensive review delves into the intricate interplay between connective tissue disorders and aortic dissection, shedding light on the clinical features, pathophysiology, genetic underpinnings, diagnostic approaches, clinical management, associated comorbidities, and prognosis, mainly focusing on MS and EDS, while also exploring rare connective tissue disorders and forms of cutis laxa contributing to aortic pathology.
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spelling pubmed-106955482023-12-05 Comprehensive review of aortic aneurysms, dissections, and cardiovascular complications in connective tissue disorders Elendu, Chukwuka Amaechi, Dependable C. Elendu, Tochi C. Ibhiedu, Jennifer O. Torubiri, Augustina O. Okoye, Osinachi K. Medicine (Baltimore) 3400 Connective tissue disorders, including Marfan syndrome (MS) and Ehlers-Danlos syndrome (EDS), are characterized by genetic mutations affecting connective tissue structural integrity. These disorders significantly elevate the risk of aortic dissection, a life-threatening condition. This comprehensive review delves into the intricate interplay between connective tissue disorders and aortic dissection, shedding light on the clinical features, pathophysiology, genetic underpinnings, diagnostic approaches, clinical management, associated comorbidities, and prognosis, mainly focusing on MS and EDS, while also exploring rare connective tissue disorders and forms of cutis laxa contributing to aortic pathology. Lippincott Williams & Wilkins 2023-12-01 /pmc/articles/PMC10695548/ http://dx.doi.org/10.1097/MD.0000000000036499 Text en Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc. https://creativecommons.org/licenses/by/4.0/This is an open access article distributed under the Creative Commons Attribution License 4.0 (CCBY) (https://creativecommons.org/licenses/by/4.0/) , which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle 3400
Elendu, Chukwuka
Amaechi, Dependable C.
Elendu, Tochi C.
Ibhiedu, Jennifer O.
Torubiri, Augustina O.
Okoye, Osinachi K.
Comprehensive review of aortic aneurysms, dissections, and cardiovascular complications in connective tissue disorders
title Comprehensive review of aortic aneurysms, dissections, and cardiovascular complications in connective tissue disorders
title_full Comprehensive review of aortic aneurysms, dissections, and cardiovascular complications in connective tissue disorders
title_fullStr Comprehensive review of aortic aneurysms, dissections, and cardiovascular complications in connective tissue disorders
title_full_unstemmed Comprehensive review of aortic aneurysms, dissections, and cardiovascular complications in connective tissue disorders
title_short Comprehensive review of aortic aneurysms, dissections, and cardiovascular complications in connective tissue disorders
title_sort comprehensive review of aortic aneurysms, dissections, and cardiovascular complications in connective tissue disorders
topic 3400
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10695548/
http://dx.doi.org/10.1097/MD.0000000000036499
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