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Linear growth of children with nephrotic syndrome: effect of alkylating agents
Insights
Children with nephrotic syndrome experienced improved growth rates after treatment with alkylating agents like cyclophosphamide or chlorambucil, reducing steroid dependence and side effects.
Area of Science:
- Pediatric Nephrology
- Pharmacology
Background:
- Steroid-dependent nephrotic syndrome often leads to growth impairment in children.
- Frequent relapses necessitate long-term immunosuppressive therapy, including steroids, which can hinder growth.
Purpose of the Study:
- To evaluate the impact of alkylating agents on growth velocity in children with frequently relapsing or steroid-dependent nephrotic syndrome.
- To assess the safety and efficacy of cyclophosphamide or chlorambucil in improving growth in this patient population.
Main Methods:
- Retrospective analysis of growth velocity in 12 children with nephrotic syndrome before and after treatment with cyclophosphamide or chlorambucil and alternate-day prednisone.
- Renal biopsies were performed prior to treatment to assess histopathological findings.
Main Results:
- Growth rates significantly increased from 4.3 cm/y before treatment to 8.7 cm/y after therapy (P < .001).
- Treatment led to diminished steroid use, with minimal short-term side effects observed.
- Despite relapses in 5 patients, overall growth velocity improved substantially.
Conclusions:
- Alkylating agents are a reasonable therapeutic option for children with nephrotic syndrome exhibiting decreased growth velocity.
- This treatment strategy can improve growth by reducing steroid exposure in children with difficult-to-manage nephrotic syndrome.
Abstract:
Growth velocity measurements were assessed in 12 children with steroid responsive but frequent relapsing or dependent nephrotic syndrome prior to and following treatment with either cyclophosphamide or chlorambucil and alternate day prednisone. Patients averaged 6 +/- 3 years (mean +/- SD) of age at the time of treatment. All of the patients underwent renal biopsy prior to treatment; 5 of the 12 showed mesangial proliferation; and in 10 of the 12, IgM deposits were seen within the mesangium of the glomerulus. Growth rates before treatment with the alkylating agent were 4.3 +/- 1.3 cm/y increasing to 8.7 +/- 2.5 cm/y (P less than .001) after therapy despite the relapses that occurred in 5 of the patients in the year following treatment. Short-term side effects of the treatment were minimal. The significant increase in growth associated with diminished use of steroids makes the use of alkylating agents reasonable for children with nephrosis who show signs of decreased growth velocity.