Sakallioglu Onur, Gok Faysal, Kalman Suleyman, Atay Abdullah Avni, Kaya Ayper, Duzova Ali, Gokcay Erdal
Pediatric Nephrology Unit, Gulhane Military Medical Academy, Etlik-Ankara, Turkey.
J Nephrol. 2006 Mar-Apr;19(2):211-4.
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.
罗萨伊-多夫曼病（R-D病）是一种淋巴系统的良性淋巴细胞组织细胞增生症。细胞因子过度表达（肿瘤坏死因子（TNF）、白细胞介素（IL）-1β和IL-6）导致的免疫紊乱被认为是R-D病的病因。我们报告一名7岁患R-D病的男孩，在R-D病进展过程中并发了微小病变性肾病（MCN）。该患者对口服泼尼松龙耐药；口服环磷酰胺（2mg/kg，共12周）后，R-D病和MCN均得到缓解。MCN是儿童肾病综合征最常见的病因，一般认为是通过细胞因子紊乱而出现的。我们病例中R-D病活动与肾病综合征的发生及缓解之间的相关性表明，肾病综合征是通过某些与R-D病相关的免疫机制诱发的。
