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Published on: November 1, 2018
Focal glomerulosclerosis in children: an Argentinian experience
A Tufro-McReddie1, E Alvarez, E Arrizurieta
1Paediatric Nephrology Section, Hospital Professor Alejandro Posadas, Buenos Aires, Argentina.
Pediatric Nephrology (Berlin, Germany)
|March 1, 1992
Summary
This study on childhood nephrotic syndrome found cyclophosphamide (CY) effective for steroid-resistant cases. Longer CY courses improved remission rates and renal function in focal glomerulosclerosis patients.
Area of Science:
- Pediatric Nephrology
- Glomerular Diseases
Background:
- Idiopathic nephrotic syndrome (INS) in children often presents with focal glomerulosclerosis (FGS).
- Steroid resistance is a significant challenge in managing childhood INS with FGS.
Purpose of the Study:
- To evaluate treatment response, clinical outcomes, and clinicopathological correlations in children with INS and FGS.
- To assess the efficacy of cyclophosphamide (CY) in steroid-resistant cases of FGS.
Main Methods:
- Retrospective study of 26 children diagnosed with INS and FGS.
- Analysis of treatment response to corticosteroids and cyclophosphamide (CY).
- Evaluation of long-term renal function and prognostic indicators.
Main Results:
- 84.6% of patients were steroid-resistant.
- Cyclophosphamide (CY) induced remission in 8/19 with focal segmental glomerulosclerosis and 2/3 with focal global glomerulosclerosis within 16 weeks.
- After 83 months follow-up, 17 patients achieved remission with normal renal function; 6 developed chronic renal failure.
Conclusions:
- Longer courses of cyclophosphamide (CY) therapy may lead to better outcomes in children with steroid-resistant focal glomerulosclerosis (FGS).
- Persistence of proteinuria, high percentage of sclerotic glomeruli, and diffuse mesangial proliferation are poor prognostic indicators.
