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Medicine (Baltimore) ; 102(48): e36499, 2023 Dec 01.
Article in English | MEDLINE | ID: mdl-38050214

ABSTRACT

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.


Subject(s)
Aortic Aneurysm , Aortic Dissection , Connective Tissue Diseases , Ehlers-Danlos Syndrome , Marfan Syndrome , Humans , Connective Tissue Diseases/complications , Marfan Syndrome/complications , Marfan Syndrome/diagnosis , Ehlers-Danlos Syndrome/complications , Ehlers-Danlos Syndrome/diagnosis , Aortic Aneurysm/complications , Aortic Dissection/etiology , Connective Tissue
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