Nephrotic syndrome- idiopathic- steroid-resistant


Nephrotic syndrome is a condition where the kidneys leak protein from the blood into the urine. Corticosteroids are used in the first instance to achieve remission. Some children do not respond to this treatment and other agents such as cyclophosphamide, chlorambucil, cyclosporin or ACE inhibitors may be used. This review found that when cyclosporin was compared to placebo or no treatment there was a significant increase in the number of children who achieved complete remission. There was no improvement with other immunosuppressive agents. However the number of studies were small


Although much of the interest in pulse methylprednisolone therapy (PMT) has centered around its use in children with focal segmental glomerulosclerosis, PMT has also been shown to be effective in the treatment of other proteinuric renal diseases. We hypothesized that a PMT-based treatment protocol, derived from the Tune-Mendoza protocol, would effectively induce a more rapid remission in young children with idiopathic steroid-resistant nephrotic syndrome (SRNS). A retrospective analysis was conducted of 11 consecutive SRNS patients (mean age 3.6 +/- 1.5 years) that received PMT between 1 August 1992 and 1 May 1998. The initial mean urinary protein/urinary creatinine ratio (UP/UC, mg/mg) was 8.3 +/- 9.7 and mean estimated creatinine clearance (CCr) 137.7 +/- 47.0 ml/min per 1.73 m2. An average of 24.8 +/- 10.5 PMT doses were given. The mean duration of PMT therapy until remission was 23.4 +/- 29.9 days (median 12 days). Cyclosporine and cyclophosphamide were used to maintain and extend remissions in 5 and 4 patients, respectively. At the conclusion of the study, the mean UP/UC was 0.12 +/- 0.22 and mean CCr 151.8 +/- 39.8 ml/min per 1.73 m2 (no CCr