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Published on: July 3, 2013
[Optimization of the treatment of idiopathic nephrotic syndrome in children]
M G Porcellini1, A Amore, B Gianoglio
1Divisione di Nefrologia e Dialisi, Ospedale Infantile Regina Margherita, Torino.
Insights
Idiopathic nephrotic syndrome in children often requires prolonged steroid therapy, with cyclophosphamide proving effective for frequent relapsers. Long-term remission is achievable for most, though a small percentage may progress to chronic renal failure.
Area of Science:
- Pediatric Nephrology
- Internal Medicine
- Clinical Research
Context:
- Idiopathic nephrotic syndrome (INS) is a significant cause of kidney disease in children.
- Understanding treatment outcomes and long-term prognosis is crucial for managing pediatric INS.
- This study reviews a cohort of children treated for INS over a decade.
Purpose:
- To analyze the treatment outcomes, relapse rates, and long-term prognosis of children with idiopathic nephrotic syndrome.
- To evaluate the efficacy of different therapeutic strategies, including steroid-sparing agents.
- To identify factors associated with disease progression and remission.
Summary:
- Eighty-one children with INS were treated, with a majority being male and diagnosed at a mean age of 4.8 years.
- Prednisone was the primary treatment, achieving a favorable response in ~90%, but half experienced frequent relapses.
- Cyclophosphamide was effective in achieving stable remission in 62% of relapsing cases; 13.6% were steroid-resistant, and 2.4% progressed to end-stage renal failure.
Impact:
- The findings highlight the effectiveness of prolonged steroid protocols and the utility of cyclophosphamide for managing relapsing cases of pediatric INS.
- Identifies a small but significant proportion of patients at risk for chronic kidney disease progression.
- Provides valuable data for optimizing treatment strategies and improving long-term outcomes for children with INS.
Abstract:
From 1981 to 1992, 81 children affected by idiopathic nephrotic syndrome were treated in our Division. The majority were males (74%), with mean age at diagnosis of 4.8 (0-14)) years. They had a mean follow-up of 5.7 (0.5-11) years. The renal function at diagnosis was normal in all cases, proteinuria was selective in 86.4% of the cases. 28 children with somehow atypical behaviour underwent renal biopsy. In this negatively selected group the more frequent histologic forms were focal segmental glomerular sclerosis (33.5%), minimal change disease (37.4%), IgM mesangial glomerulonephritis (16.6%), membranous nephropathy (12.5%). First choice drug was in all cases prednisone. In the last years the trend for steroid therapy was to adopt long protocols (16 weeks), with a total dose of 105-133 mg/kg per therapeutic cycle. About 90% of children had a favourable response to steroids, but half of them were frequent relapser. In these cases the long term stable remission was obtained with steroids alone in 10.8% of the cases, with the association of cyclophosphamide in 62%. In a minor fraction and in the steroid resistant (13.6% of the total cases) levamisol and cyclosporin were also adopted. 2.4% of the cases progressed to end stage chronic renal failure. Most of the steroid sensitive children were in remission after 3-4 years of disease, only 6% relapsed more than 5 years after diagnosis.
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