Article
Proposed Stages of Myocardial Phenotype Development in Fabry Disease.
JACC. Cardiovascular imaging - 1 Aug 2019
Nordin Sabrina, Kozor Rebecca, Medina-Menacho Katia, Abdel-Gadir Amna, Baig Shanat, Sado Daniel M, Lobascio Ilaria, Murphy Elaine, Lachmann Robin H, Mehta Atul, Edwards Nicola C, Ramaswami Uma, Steeds Richard P, Hughes Derralynn, Moon James C
Abstract excerpt
OBJECTIVES: This study sought to explore the Fabry myocardium in relation to storage, age, sex, structure, function, electrocardiogram changes, blood biomarkers, and inflammation/fibrosis. BACKGROUND: Fabry disease (FD) is a rare, x-linked lysosomal storage disorder. Mortality is mainly cardiovascular with men exhibiting cardiac symptoms earlier than women. By cardiovascular magnetic resonance, native T1 is low...
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