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Mesangial proliferative glomerulonephritis in children

M Uszycka-Karcz, J Stolarczyk, T Wrzolkowa

    The International Journal of Pediatric Nephrology
    |December 1, 1982
    PubMed
    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.

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    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.

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  • 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.