Isolated aortic root dilation in homocystinuria
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چکیده
منابع مشابه
Angiotensin II blockade and aortic-root dilation in Marfan's syndrome.
BACKGROUND Progressive enlargement of the aortic root, leading to dissection, is the main cause of premature death in patients with Marfan's syndrome. Recent data from mouse models of Marfan's syndrome suggest that aortic-root enlargement is caused by excessive signaling by transforming growth factor beta (TGF-beta) that can be mitigated by treatment with TGF-beta antagonists, including angiote...
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Aortic root dilation is an important, potentially lethal omplication of BAV (4) that substantially increases the risk f ascending aortic aneurysm, dissection, and rupture (3). uriously, aortic valve replacement does not prevent proressive dilation of the ascending aorta (5), suggesting that echanisms unrelated to hemodynamic factors, or to the alve per se, contribute to the aortopathy of BAV. A...
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ژورنال
عنوان ژورنال: Journal of Inherited Metabolic Disease
سال: 2017
ISSN: 0141-8955,1573-2665
DOI: 10.1007/s10545-017-0094-7