Renal involvement in Neimann-Pick Disease

Carrie A. Grafft, Fernando C. Fervenza, Merfake H. Semret, Sheldon Orloff, Sanjeev Sethi

Research output: Contribution to journalArticlepeer-review

12 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)448-451
Number of pages4
JournalNDT Plus
Issue number6
StatePublished - Dec 2009


  • Neimann-Pick disease
  • myelin inclusions
  • zebra bodies

ASJC Scopus subject areas

  • Nephrology
  • Transplantation


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