Renal Pathology in Fabry Disease

Figure 2. Electron microscopy in Fabry disease. (A) Electron microscopy of a renal biopsy obtained from a 26-yr-old male Fabry patient demonstrates accumulation of storage material within the secondary lysosomes of podocytes and glomerular parietal epithelial cells. There is focal foot process effacement. There is free glycolipid in the urinary space and a thickened Bowman’s capsule (magnification, ×3760). (Inset) Higher magnification demonstrates enlarged lysosomes within a podocyte and a mixture of lamellated membrane structures and small vesicles in the glomerular parietal epithelium (magnification, ×11,200). (B) Electron microscopy of renal biopsy obtained from a male Fabry patient shows a small artery, with lysosomal storage located within endothelial cells (E) and smooth muscle cells (S) (magnification, ×3800). (Inset) Higher magnification of the endothelial cell cytoplasm demonstrates enlarged secondary lysosomes packed with storage material, which has a somewhat heterogeneous appearance (magnification, ×11,200).