The distribution of hypertrophy in anderson fabry disease
نویسندگان
چکیده
منابع مشابه
The distribution of hypertrophy in anderson fabry disease
Background AFD is an X linked disorder of lysosomal storage that can have various cardiac manifestations, most commonly resulting in LVH. Echocardiographic data on LVH distribution emphasised concentric hypertrophy in males and later onset in females with less hypertrophy. However this modality is often limited in visualisation of the left ventricular apex. Methods 27 patients (mean age 47, 33%...
متن کاملAnderson-Fabry disease: extrarenal, neurologic manifestations.
The advent of enzyme replacement therapy for AndersonFabry disease (AFD) adds impetus for the early detection of patients with this inherited multiorgan lipid storage disease. The resultant accumulation of neutral glycosphingolipids, especially globotriaosylceramide (Gb3), in various cell types promotes development of disease-related complications associated with renal, cardiovascular, and cere...
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BACKGROUND Anderson-Fabry disease is an X-linked defect of glycosphingolipid metabolism. Progressive renal insufficiency is a major source of morbidity, additional complications result from cardio- and cerebro-vascular involvement. Survival is reduced among affected males and symptomatic female carriers. OBJECTIVES To evaluate the effectiveness and safety of enzyme replacement therapy compare...
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Anderson-Fabry disease (AFD) is an X-linked storage disorder caused by a deficiency of the lysosomal hydrolase a-galactosidase A (AGAL) and the resultant accumulation of its glycosphingolipid substrate (Gb3) in several tissue types. Major morbidity and reduced life expectancy among affected individuals are a consequence of renal, cardiac and cerebrovascular involvement. Symptomatic males and fe...
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A case of basilar artery aneurysm is described. The patient had a family history of similar aneurysms and also of a rare spingolipidosis, Anderson-Fabry disease.
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ژورنال
عنوان ژورنال: Journal of Cardiovascular Magnetic Resonance
سال: 2011
ISSN: 1532-429X
DOI: 10.1186/1532-429x-13-s1-p278