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Steroid-resistant, cyclosporine-responsive, relapsing nephrotic syndrome
1Department of Pediatrics, Emory University School of Medicine, Atlanta, Georgia 30322, USA.
Pediatric Nephrology (Berlin, Germany)
|April 1, 1995
Summary
Cyclosporine A (CsA) effectively treated pediatric steroid-resistant nephrotic syndrome, but relapses and chronic kidney disease remain significant concerns requiring long-term management.
Area of Science:
- Pediatric Nephrology
- Immunosuppressive Therapy
- Glomerular Diseases
Background:
- Steroid-resistant nephrotic syndrome (SRNS) presents a significant therapeutic challenge in children.
- Limited effective treatment options exist for SRNS, often leading to poor renal outcomes.
Purpose of the Study:
- To evaluate the efficacy and safety of cyclosporine A (CsA) in children with SRNS.
- To assess remission rates, renal function, and relapse patterns during and after CsA therapy.
Main Methods:
- Eighteen children with SRNS (minimal change disease, focal segmental glomerulosclerosis, mesangial hypercellularity) received CsA.
- Doses were adjusted to maintain whole blood trough levels of 80-200 ng/ml.
- Patients were monitored for remission, serum creatinine, and relapse occurrence.
Main Results:
- Fourteen of 18 patients achieved complete or partial remission within 2 months.
- Serum creatinine remained stable in 17 patients during CsA therapy.
- Relapses occurred in 9 patients after CsA discontinuation, with most responding to re-treatment; however, 4 patients developed chronic renal failure.
Conclusions:
- Initial CsA therapy is effective for pediatric SRNS, achieving high remission rates.
- CsA dependency, frequent relapses, and the risk of chronic renal failure necessitate careful long-term monitoring and management strategies.