Fabry cardiomyopathy: Gb3‐induced auto‐reactive panmyocarditis requiring heart transplantation
نویسندگان
چکیده
منابع مشابه
Fabry Cardiomyopathy
Fabry disease is a progressive X-linked disorder of glycosphingolipid metabolism caused by a deficiency of the α-galactosidase lysosomal enzyme. The partial or complete deficiency of the lysosomal enzyme leads to an accumulation of neutral glycosphingolipids in the vascular endothelium and visceral tissues throughout the body. In the heart, glycosphingolipids deposition causes progressive left ...
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متن کاملA Case of Fabry Cardiomyopathy
In the absence of hypertension, hypertrophic cardiomyopathy is the most common cause of left ventricular hypertrophy (LVH). However, it has been reported that up to 3% of males with unexplained LVH have Fabry disease, an X-linked disorder of glycophospholipid metabolism that is due to a deficiency in the lysosomal enzyme alpha-galactosidase A (alpha-Gal A). A 44-year-old man was admitted to our...
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ژورنال
عنوان ژورنال: ESC Heart Failure
سال: 2020
ISSN: 2055-5822,2055-5822
DOI: 10.1002/ehf2.12723