We describe the renal biopsy findings in a 14-year-old girl with Neimann-Pick disease. The renal biopsy showed chronic changes involving all components of the parenchyma, including focal global glomerulosclerosis, tubular atrophy, interstitial fibrosis and vascular sclerosis. On light microscopy, significant findings included foamy podocytes, vacuolated tubular epithelial cells and collections of foam cells in the interstitium. Electron microscopy was confirmatory which showed myelin-like inclusions in podocytes, endothelial cells, tubular epithelial cells and small nerves. The findings are similar to Fabry's disease, except that small nerve involvement appears to be unique to Neimann Pick disease.
Keywords: Neimann-Pick disease; myelin inclusions; zebra bodies.