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Published on: May 6, 2018
Management of the difficult nephrotic patient
1Department of Pediatrics, University of California, School of Medicine, San Diego, USA.
Most children with nephrotic syndrome experience relapses and remissions. Steroid-resistant cases, often focal segmental glomerulosclerosis, may benefit from cyclosporine or specific treatment protocols to improve outcomes and prevent kidney failure.
Area of Science:
- Pediatric Nephrology
- Glomerular Diseases
Background:
- Nephrotic syndrome is common in children, often managed with steroids.
- Some children develop steroid resistance and toxicity, necessitating alternative treatments.
- Focal segmental glomerulosclerosis (FSGS) is a frequent biopsy finding in steroid-resistant nephrotic syndrome.
Purpose of the Study:
- To review management strategies for children with nephrotic syndrome.
- To identify effective treatments for steroid-resistant nephrotic syndrome, particularly FSGS.
- To explore options for patients with significant steroid toxicity.
Main Methods:
- Review of clinical management protocols for nephrotic syndrome.
- Analysis of treatment outcomes for steroid-resistant cases.
- Evaluation of therapeutic agents including alkylating agents, cyclosporine, and pulse methylprednisolone.
Main Results:
- Many children with nephrotic syndrome achieve remission with standard steroid therapy.
- Steroid-resistant nephrotic syndrome, often linked to FSGS, poses a challenge.
- Alternative treatments like oral alkylating agents or cyclosporine can manage some steroid-dependent cases.
- Pulse intravenous methylprednisolone and oral alkylating agents may improve outcomes in steroid-resistant FSGS.
Conclusions:
- While most children with nephrotic syndrome respond to steroids, a subset requires alternative therapies.
- Cyclosporine and specific combination therapies show promise for managing steroid-resistant nephrotic syndrome and FSGS.
- Effective management of steroid resistance is crucial to prevent progression to chronic kidney disease.
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