Denys-Drash syndrome

 

 

This 21 month old boy with hypospadias and undescended testicles underwent renal transplant because of

 end-stage renal failure.  This section is from one of his end-stage kidneys. There is diffuse sclerosis with

secondary vascular changes.  The glomeruli are almost entirely sclerosed.  EM of the least involved glomeruli

showed marked increase of mesangial matrix and subepithelial electron-dense deposits.

 

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