Marfan syndrome: A therapeutic challenge for long-term care
Abstract
Marfan syndrome (MFS) is an autosomal dominant genetic disorder caused by mutations in the fibrillin-1 gene. Acute aortic dissection is the leading cause of death in patients suffering from MFS and consequence of medial degeneration and aneurysm formation. In addition to its structural function in the formation of elastic fibers, fibrillin has a major role in keeping maintaining transforming growth factor β (TGF-β) in an inactive form....
Paper Details
Title
Marfan syndrome: A therapeutic challenge for long-term care
Published Date
Jun 1, 2019
Journal
Volume
164
Pages
53 - 63
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Notes
History