سیر بالینی، ریسک فاکتورهای عود و پیامد طولانی مدت کودکان مبتلا به سندرم نفروتیک اولیه، مرکز طبی کودکان، 79-1360

The nephrotic syndrome is the most common chronic renal disease of childhood.Materials and Methods: In this study the clinical course, risk factors for relapse and the predictors of long-term outcome of 502 patients (median age 5 years)with primary nephrotic syndrome were followed for an average of 60 months (3.5 to 240 months) from 1981 to 2000.Results: Among the 502 patients 5 (1%) achieved spontaneous remission and 313 children were initial responder. One hundred eighty four patients received at least 1 kidney biopsy (78 prior and 106 after initiation of treatment). Of 104 children with frequently relapsing steroid sensitive and steroid dependent nephrotic syndrome, levamisole induced prolong remission in 33 ( 31.7%) of patients. Cyclophosphamid and cyclosporine A induced prolong remission in 49 (50%) of 98 and 28 (41.3%) of 68 patients respectively. At the time of the final clinical evaluation, 73 patients (14.5%) were on remission 301 (59.9%) had relapsing 43 (8.6%) had persistent nephrotic syndrome 33 (6.6%) of patients evolving to end-stage renal disease (ESRD) and 6 (1.2%) of them with chronic renal failure died (infection and cardio respiratory were the cause of death in 5 and 1 patient respectively). Young age (1-5 y) at onset of disease and atopy were identified as an independent risk factors for relapse (P0.05). Patients with steroid dependent nephrotic syndrome (SDNS) or MCNS had better response to cyclophosphamide or cyclosporin than children with steroid resistance nephrotic syndrome (SRNS) or FSGS (P0.05). Persistent proteinuria, hypertension, microscopic or macroscopic hematuia, glucosuria were associated with progression to chronic renal failure (PO.05).Conclusion: Steroid dependency and histopathology of MCNS in patients with nephrotic syndrome were significantly associated with good long-term prognosis. In contrast persistent proteinuria, histopatholoy of FSGS, hypertension, macroscopic or microscopic hematuria, and glucosoria were significantly correlated with unfavorable long-term outcome. Additionally our study showed a positive correlation between young age and atopy with higher rate of relapse.