Thursday, February 17, 2011


Image Quiz!!!
What kidney disease do you see the above findings on Electron Microscopy?

Interferon associated FSGS
  5 (7%)
  4 (5%)
  1 (1%)
Fabry's Disease
  54 (79%)
Adenovirus Nephritis
  4 (5%)

The vast majority of you said Fabry's Disease.
That is correct.
In HIV, HTLV and Interferon FSGS, one would see Tubular reticular inclusions. In Adenovirus Nephritis, sometimes, you can catch the virus in the cell on EM that might be looking like above.
The above is a Zebra body classically seen in Fabry's Disease. It is an X linked disease that is a lysosomal storage disease and deficiency in activity of alpha galactosidase.
The RFN has a nice recent blog on the disease inheritance.
What does one see on Kidney Biopsy?
1 Light Microscopy: foamy visceral epithelial cells. vacuoles in podocytes, mesangial widening, most of this lipid material deposits in the podocytes and some in epithelial and then endothelial cells. Advanced disease can show FSGS as well.
2. Electron microscopy: enlarged secondary lysosomes (zebra bodies as shown above) packed with lamellated membrane structures.  As diseases advances, fusion of foot processes and proteinuria. 
A nice paper on pathology in the kidney is in JASN. look below


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