Minimal change nephropathy in a 7-year-old boy with Rosai-Dorfman disease.
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.
Sakallioglu
Onur
O
Pediatric Nephrology Unit, Gulhane Military Medical Academy, Etlik-Ankara, Turkey. onursakallioglu@hotmail.com
Gok
Faysal
F
Kalman
Suleyman
S
Atay
Abdullah Avni
AA
Kaya
Ayper
A
Duzova
Ali
A
Gokcay
Erdal
E
D000284:Administration, Oral; D000893:Anti-Inflammatory Agents / Q000008:administration & dosage; D002648:Child; D003520:Cyclophosphamide / Q000008:administration & dosage*; D016207:Cytokines / Q000276:immunology; D018450:Disease Progression; D004351:Drug Resistance / Q000187:drug effects / Q000276:immunology; D015618:Histiocytosis, Sinus / Q000150:complications / Q000188:drug therapy* / Q000276:immunology / Q000473:pathology; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage*; D008297:Male; D009402:Nephrosis, Lipoid / Q000188:drug therapy* / Q000209:etiology / Q000276:immunology / Q000473:pathology; D011239:Prednisolone / Q000008:administration & dosage
D000893:Anti-Inflammatory Agents; D016207:Cytokines; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D011239:Prednisolone