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Published on: May 6, 2018
Steroid resistant nephrotic syndrome is sustained remission attainable
B R Nammalwar1, M Vijaykumar, N Prahlad
1Division of Nephrology, Kanchi Kamakoti CHILDS Trust Hospital, 12A, Nageswar Road, Nungambakkam, Chennai 600 034, Tamilnadu, India. drmvk@vsnl.net
Insights
Immunosuppressive therapy shows promise for steroid-resistant nephrotic syndrome in children. This treatment effectively induced remission in minimal change disease and diffuse mesangial proliferation but was less effective for focal segmental glomerulosclerosis.
Area of Science:
- Pediatric Nephrology
- Immunosuppressive Therapy
- Glomerular Diseases
Background:
- Steroid-resistant nephrotic syndrome (SRNS) presents a significant therapeutic challenge in pediatric nephrology.
- Identifying effective immunosuppressive regimens for SRNS is crucial for improving patient outcomes.
Purpose of the Study:
- To evaluate the efficacy of a specific immunosuppressive protocol in children diagnosed with steroid-resistant nephrotic syndrome.
- To assess the long-term remission rates across different histopathological subtypes of SRNS.
Main Methods:
- A prospective study involving 34 children with SRNS.
- Treatment consisted of intravenous methylprednisolone, oral prednisolone for one year, and six monthly pulses of intravenous cyclophosphamide.
- Disease remission was monitored at one, two, and three years post-treatment.
Main Results:
- The protocol achieved remission in 81.8% of children with minimal change nephrotic syndrome (MCNS) at three years.
- Remission rates were 66.7% for diffuse mesangial proliferation (DMP) and only 16.7% for focal segmental glomerulosclerosis (FSGS).
- The therapy demonstrated significant benefit in MCNS and DMP subtypes.
Conclusions:
- Intravenous methylprednisolone, oral prednisolone, and cyclophosphamide pulse therapy is beneficial for pediatric SRNS with MCNS and DMP.
- This immunosuppressive regimen is not effective for children with focal segmental glomerulosclerosis.
- Further research may be needed to explore alternative treatments for SRNS in FSGS cases.
Abstract:
A prospective study was undertaken to find out the benefit of immunosuppressive therapy(IV methyl prednisolone followed by oral prednisolone therapy for one year along with six doses of pulse monthly IV cyclophosphamide) in children with steroid resistant nephrotic syndrome. Thirty-four children with steroid resistant nephrotic syndrome were treated with above regime. The remission of the disease was determined at the end of first, second and third year. The above protocol could induce and maintain remission in 81.8% of children with minimal change nephrotic syndrome, 66.7% of children with diffuse mesangial proliferation and in only 16.7% of children with focal segmental glomerulosclerosis at the end of three years of the study. The therapy of IV methyl prednisolone followed by oral prednisolone for one year with 6 monthly pulse IV cyclophosphamide, is beneficial in children with steroid resistant minimal change disease and diffuse mesangial proliferative glomerulonephritis. The therapy is not effective in focal segmental glomerulosclerosis.
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