Figure 36

Electron microscopy of nail-patella syndrome. This disorder having skeletal and renal manifestations affects the glomeruli, with accumulation of banded collagen fibrils within the substance of the capillary basement membrane. This accumulation appears as empty lacunae when the usual stains with electron microscopy (lead citrate and uranyl acetate) are used. However, as here, the fibrils easily can be identified with the use of phosphotungstic acid stain in conjunction with or instead of typical stains. Note that this disorder differs structurally from collagen type III glomerulopathy in which the collagen fibrils are subendothelial and not intramembra-nous in location. Patients with nail-patella syndrome may develop proteinuria, sometimes in the nephrotic range, with variable progression to end-stage renal failure. No distinguishing abnormalities are seen on light microscopy.

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