AI Article Synopsis
- Connective tissue disorders like Marfan syndrome (MS) and Ehlers-Danlos syndrome (EDS) are caused by genetic mutations that weaken connective tissue, increasing the risk of serious complications like aortic dissection.
- This review examines how these disorders relate to aortic dissection, covering clinical symptoms, underlying biological mechanisms, genetic factors, diagnosis, treatment strategies, and possible related health issues.
- The focus is primarily on MS and EDS, but it also includes insights into less common connective tissue disorders and conditions like cutis laxa that may lead to aortic problems.
Article 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.
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Source |
|---|---|
| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC10695548 | PMC |
| http://dx.doi.org/10.1097/MD.0000000000036499 | DOI Listing |
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