MP093A SIMPLIFIED CLINICAL PRE-HISTOLOGIC SCORING METHOD OF KIDNEY BIOPSIES IN FABRY DISEASE
نویسندگان
چکیده
منابع مشابه
Fabry Disease Chronic Kidney Disease
Fabry disease (FD) arises from an X-linked defect in lipid storage, whereby deficient or absent lysosomal α-galactosidase A (α-gal A) activity leads to systemic deposition of glycosphingolipids, mainly globotriaosylceramide (known as Gb3 or GL3). Deposition mainly affects the cardiovascular, renal, and neurologic systems, but can occur in all organs, and despite specific enzyme replacement ther...
متن کاملThe Kidney in Fabry Disease
Fabry disease is a rare cause of end-stage renal disease. Renal pathology is notable for diffuse deposition of glycosphingolipid in the renal glomeruli, tubules, and vasculature. Classical patients with mutations in the a-galactosidase A gene accumulate globotriaosylceramide and become symptomatic in childhood with pain, gastrointestinal disturbances, angiokeratoma, and hypohidrosis. Classical ...
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ژورنال
عنوان ژورنال: Nephrology Dialysis Transplantation
سال: 2017
ISSN: 0931-0509,1460-2385
DOI: 10.1093/ndt/gfx162.mp093