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Mesangial proliferative glomerulonephritis in children
Summary
Cyclophosphamide (CP) effectively treated pediatric nephrotic syndrome (NS), inducing remission in most patients. Steroid therapy showed limited long-term efficacy for children with mesangial proliferative glomerulonephritis.
Area of Science:
- Pediatric Nephrology
- Glomerular Diseases
- Immunosuppressive Therapy
Background:
- Mesangial proliferative glomerulonephritis (MsPGN) is a significant cause of kidney disease in children.
- Nephrotic syndrome (NS) and nephritic syndrome are common clinical presentations of MsPGN.
- Steroid therapy is a first-line treatment for pediatric NS, but response rates and remission durations vary.
Purpose of the Study:
- To evaluate the efficacy of cyclophosphamide (CP) in children with MsPGN presenting with nephrotic syndrome.
- To compare the outcomes of CP treatment with steroid therapy in this pediatric population.
- To assess the long-term renal outcomes in children with MsPGN presenting with nephritic syndrome.
Main Methods:
- Retrospective analysis of 105 children diagnosed with MsPGN over a ten-year period.
- Patients were categorized into two groups: those presenting with NS and those with nephritic syndrome/proteinuria.
- Treatment outcomes, including remission rates, relapse frequency, and response to steroids and CP, were analyzed.
Main Results:
- Of 92 children with NS treated with steroids, 26 showed no response, and 56 had short remissions with frequent relapses.
- Cyclophosphamide (CP) treatment in 89 children with NS (including steroid-resistant and steroid-dependent cases) resulted in remission in 84 patients, with a mean remission duration of 46 months.
- Only 5 children did not respond to CP. No correlation was found between histological changes and treatment response.
- The 13 children with nephritic syndrome/proteinuria remained untreated, with no observed progression of renal disease over several years.
Conclusions:
- Cyclophosphamide (CP) demonstrates high efficacy in achieving prolonged remission in pediatric nephrotic syndrome (NS) associated with mesangial proliferative glomerulonephritis (MsPGN).
- Steroid therapy has limited long-term effectiveness for MsPGN, characterized by frequent relapses and non-response.
- Children with MsPGN presenting with nephritic syndrome may have a benign course without specific treatment.