Pearl of the Day “The standard medical therapy for patients with Marfan syndrome and Loeys-Dietz syndrome is β-blockers . Recent research has shown that mutations underlying both Marfan and Loeys-Dietz syndromes result in dysregulation of the TGF-β signaling pathway. This finding led to hopes that losartan, an angiotensin II receptor blocker that can significantly attenuate TGF-β signaling, may be able to alter the natural history of Marfan and Loeys-Dietz syndromes. Losartan has been shown to decrease the rate of progression of aortic root dilatation in mouse models of Marfan syndrome and in a small observational cohort of patients with Marfan syndrome. Currently, several randomized controlled trials are in progress to evaluate the effectiveness of losartan in delaying the aortic root growth rate in patients with Marfan syndrome.” CT Angiographic Evaluation of Genetic Vascular Disease: Role in Detection, Staging, and Management of Complex Vascular Pathologic Conditions Chu LC , Johnson PT, Dietz HC, Fishman EK AJR 2014; 202:1120–1129
Posted on: Sat, 29 Nov 2014 12:00:00 +0000
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