Electrocardiographic Changes and Arrhythmia in Fabry Disease
نویسندگان
چکیده
منابع مشابه
Electrocardiographic Changes and Arrhythmia in Fabry Disease
Fabry disease is an X-chromosome-linked lysosomal storage disease characterized by a deficient activity or, in most males, absence of the enzyme α-galactosidase A (a-Gal A) leading to systemic, primary lysosomal accumulation of globotriaosylceramide (Gb3) (1). Recent literature refers to an overall birth prevalence of 1:40,000-170,000; however, such data do not allow an estimation on an actual ...
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BACKGROUND AND PURPOSE Dolichoectasia of the basilar artery is a characteristic finding of Fabry disease. However, its prevalence, severity, and course have been poorly studied. This study quantitatively evaluated, by MRA, a panel of basilar artery parameters in a large cohort of patients with Fabry disease. MATERIALS AND METHODS Basilar artery mean diameter, curved length, "origin-to-end" li...
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Fabry disease is a rare X-linked inborn error of the glycosphingolipid metabolism caused by deficient activity of lysosomal enzyme alpha-galactosidase A. It is characterized by progressive multisystemic involvement that leads to premature death due to major organ failure, particularly the kidneys and heart. It appears that the disease is underdiagnosed in patients with end-stage renal disease. ...
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ژورنال
عنوان ژورنال: Frontiers in Cardiovascular Medicine
سال: 2016
ISSN: 2297-055X
DOI: 10.3389/fcvm.2016.00007