Elevated Endothelial Microparticles in Fabry Children Decreased After Enzyme Replacement Therapy
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چکیده
منابع مشابه
Elevated endothelial microparticles in Fabry children decreased after enzyme replacement therapy.
Elevated Endothelial Microparticles in Fabry Children Decreased After Enzyme Replacement Therapy To the Editor: Fabry disease (FD), an X-linked metabolic disorder, is caused by insufficient activity of the lysosomal enzyme alpha galactosidase A ( -gal A). This results in impaired catabolism of globotriaosylceramide ([Gb3] also called ceramidetrihexoside) and its subsequent accumulation leading ...
متن کاملEnzyme replacement therapy for Anderson-Fabry disease.
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...
متن کاملEnzyme replacement therapy and Fabry nephropathy.
Involvement of the kidneys in Fabry disease ("nephropathy") occurs in male and female individuals. The majority of patients with progressive nephropathy will have significant proteinuria and develop progressive loss of kidney function, leading to ESRD. All too often, treating physicians may ignore "normal" serum creatinine levels or "minimal" proteinuria and fail to assess properly the severity...
متن کاملAnderson-Fabry disease: enzyme replacement therapy.
Sir, Anderson-Fabry disease is a metabolic lysosomal storage disease caused by a deficiency of the enzyme a-galactosidase A and inherited as an X-linked recessive trait. The progressive accumulation of glycosphingolipids (globotriaosylceramide, GB3) in blood, vessels and cells from several organs and tissues causes significant multi-systemic damage in homozygous males and in carrier females. Fo...
متن کاملElevated cerebral blood flow velocities in Fabry disease with reversal after enzyme replacement.
BACKGROUND AND PURPOSE Fabry disease is an X-linked inherited disorder resulting from a deficiency of alpha-galactosidase A. Cerebrovascular disease in Fabry disease includes small-vessel disease and larger-vessel ectasia in a predominantly posterior distribution. We assessed transcranial Doppler (TCD) blood flow velocities in naive and enzyme-treated Fabry patients. METHODS TCD was used to n...
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ژورنال
عنوان ژورنال: Arteriosclerosis, Thrombosis, and Vascular Biology
سال: 2007
ISSN: 1079-5642,1524-4636
DOI: 10.1161/atvbaha.107.143511