Minimal change nephropathy in a 7-year-old boy with Rosai-Dorfman disease

J Nephrol. Mar-Apr 2006;19(2):211-4.

Abstract

Rosai-Dorfman (R-D) disease is a benign lympho-histiocytosis of the lymphoid system. Immune derangement due to cytokine over-expression (tumor necrosis factor (TNF), interleukin (IL)-1b and IL-6) has been considered the cause of R-D disease. We present a 7-year-old boy with R-D disease who developed minimal change nephropathy (MCN) during the progression of R-D disease. The patient was resistant to oral prednisolone; and the remission of both R-D disease and MCN was achieved with oral cyclophosphamide (2 mg/kg, 12 weeks). MCN, the most common cause of nephrotic syndrome in childhood, is generally accepted to emerge by way of cytokine derangement. Correlation between R-D disease activity and the development and remission of nephrotic syndrome in our case suggested that nephrotic syndrome had been induced through some R-D disease-related immune mechanisms.

Publication types

  • Case Reports

MeSH terms

  • Administration, Oral
  • Anti-Inflammatory Agents / administration & dosage
  • Child
  • Cyclophosphamide / administration & dosage*
  • Cytokines / immunology
  • Disease Progression
  • Drug Resistance / drug effects
  • Drug Resistance / immunology
  • Histiocytosis, Sinus / complications
  • Histiocytosis, Sinus / drug therapy*
  • Histiocytosis, Sinus / immunology
  • Histiocytosis, Sinus / pathology
  • Humans
  • Immunosuppressive Agents / administration & dosage*
  • Male
  • Nephrosis, Lipoid / drug therapy*
  • Nephrosis, Lipoid / etiology
  • Nephrosis, Lipoid / immunology
  • Nephrosis, Lipoid / pathology
  • Prednisolone / administration & dosage

Substances

  • Anti-Inflammatory Agents
  • Cytokines
  • Immunosuppressive Agents
  • Cyclophosphamide
  • Prednisolone