Background:Idiopathic nephrotic syndrome is the most common glomerular disease in children.This study was undertaken to observe the efficacy and side-effects of rituximab(RTX)in treating children with different types ...Background:Idiopathic nephrotic syndrome is the most common glomerular disease in children.This study was undertaken to observe the efficacy and side-effects of rituximab(RTX)in treating children with different types of refractory primary nephrotic syndrome.Methods:Twelve patients with steroid dependent nephrotic syndrome(SDNS),frequently relapsing nephritic syndrome(FRNS),and steroid resistant nephrotic syndrome(SRNS)were enrolled in our study.There were obvious drug side-effects,and proteinuria remained difficult to control.RTX was administered at a dose of 375 mg/m2 body surface area,once or twice weekly.Results:The male to female ratio was 3:1,and the onset age was 1.6–8.9 years.There were 9 patients with steroid sensitive nephrotic syndrome(SDNS or FRNS),and 3 patients with SRNS.There were 7 patients with minimal change disease(MCD),3 patients with focal segmental glomerular sclerosis(FSGS),1 with focal proliferative glomerulonephritis,and 1 without renal biopsy.The total effective treatment rate of RTX was 91.67%,and for 77.78%of the patients,steroid dosage could be reduced.Six months before and after RTX infusion,the mean steroid dosage was significantly decreased(P=0.014)and the recurrence number was significantly reduced(P<0.001).The results were better in MCD patients than in FSGS patients(P=0.045).There was no significant difference between FRNS/SDNS and SRNS patients(P=0.175).During RTX administration,3 patients developed skin rashes,1 developed hypotension,and 1 developed a fever.One patient experienced a persistent decrease in serum immunoglobulin level but without serious infection.Conclusion:RTX was effective in the treatment of refractory nephrotic syndrome,and it could significantly reduce the use of steroid and immunosuppressants.展开更多
文摘Background:Idiopathic nephrotic syndrome is the most common glomerular disease in children.This study was undertaken to observe the efficacy and side-effects of rituximab(RTX)in treating children with different types of refractory primary nephrotic syndrome.Methods:Twelve patients with steroid dependent nephrotic syndrome(SDNS),frequently relapsing nephritic syndrome(FRNS),and steroid resistant nephrotic syndrome(SRNS)were enrolled in our study.There were obvious drug side-effects,and proteinuria remained difficult to control.RTX was administered at a dose of 375 mg/m2 body surface area,once or twice weekly.Results:The male to female ratio was 3:1,and the onset age was 1.6–8.9 years.There were 9 patients with steroid sensitive nephrotic syndrome(SDNS or FRNS),and 3 patients with SRNS.There were 7 patients with minimal change disease(MCD),3 patients with focal segmental glomerular sclerosis(FSGS),1 with focal proliferative glomerulonephritis,and 1 without renal biopsy.The total effective treatment rate of RTX was 91.67%,and for 77.78%of the patients,steroid dosage could be reduced.Six months before and after RTX infusion,the mean steroid dosage was significantly decreased(P=0.014)and the recurrence number was significantly reduced(P<0.001).The results were better in MCD patients than in FSGS patients(P=0.045).There was no significant difference between FRNS/SDNS and SRNS patients(P=0.175).During RTX administration,3 patients developed skin rashes,1 developed hypotension,and 1 developed a fever.One patient experienced a persistent decrease in serum immunoglobulin level but without serious infection.Conclusion:RTX was effective in the treatment of refractory nephrotic syndrome,and it could significantly reduce the use of steroid and immunosuppressants.
