INTERVENTIONS FOR STEROID RESISTANT IDIOPATHIC NEPHROTIC SYNDROME IN CHILDREN: A SYSTEMATIC REVIEW

Habashy D, Hodson EM, Craig JC.
Centre for Kidney Research, Children’s Hospital at Westmead, Locked Bag 4001, Westmead, NSW 2145 and Department of Public Health and Community Medicine, University of Sydney, Sydney, NSW, Australia

The optimal treatment of children with steroid resistant idiopathic nephrotic syndrome (SRINS) remains uncertain. The aim of this systematic review of randomised controlled trials (RCTs) was to evaluate the benefits and harms of different interventions used in children with SRINS, who did not achieve remission following four weeks or more of daily steroid therapy.

Using optimally sensitive search strategies, 9 eligible RCTs were identified from Medline, Embase and the Cochrane Controlled Trials Registry. The trials were small and of variable quality; 4 trials reported adequate allocation concealment and 3 reported blinding of patients and investigators.

Cyclosporin significantly reduced the number of children who failed to achieve complete remission, compared with placebo or no treatment [3 trials; 49 children; relative risk (RR) 0.64, 95% confidence intervals (CI), 0.47 to 0.88]. There was no evidence that oral cyclophosphamide [2 trials; 88 children; RR 1.01, 95% CI 0.74 to 1.36] or azathioprine [1 trial; 21 children; RR 1.01, 95% CI 0.77 to 1.32] reduced the number of children with persistent nephrotic syndrome. Intravenous cyclophosphamide reduced the number of children with persistent proteinuria but patient numbers were too small to demonstrate a significant effect (1 trial; 11 children; RR 0.09, 95% CI, 0.01 to 1.39). In a cross over trial (25 children), enalapril 0.6mg/kg/day but not enalapril 0.2mg/kg/day reduced albuminuria significantly. Tuna fish oil (1 crossover trial; 5 children) had no effect on proteinuria. No RCTS were identified, which examined the benefits and harms of high dose intravenous prednisolone with alkylating agents or cyclosporin. Cyclosporin and intravenous cyclophosphamide may be of some benefit in treating children with SRINS. Further properly designed and adequately powered trials are required to assess these and other interventions in children with SRINS.

Presented at the Australian and New Zealand Society of Nephrology, Annual Meeting, Sydney Convention Centre, 2 - 4 Sept 2002 Sydney NSW.

Correspondence
Elizabeth Hodson
Centre for Kidney Research
The Children’s Hospital at Westmead
Locked Bag 4001
Westmead NSW 2145 Sydney Australia
Tel: +61 2 9845 3430
Email1:Elizabeth Hodson