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Related Experiment Videos

Nephrotic syndrome in children

P Niaudet1

  • 1Service de Nephrologie Pediatrique, Hôpital Necker Enfants-Malades, Paris, France.

Current Opinion in Pediatrics
|April 1, 1993
PubMed
Summary

Idiopathic nephrotic syndrome in children involves glomerular charge loss. Corticosteroids are primary treatment, with relapses common, but other therapies like cyclosporine show promise for specific patient groups.

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Area of Science:

  • Pediatric Nephrology
  • Glomerular Diseases
  • Immunology

Background:

  • Idiopathic nephrotic syndrome (INS) in children is characterized by proteinuria due to glomerular basement membrane charge selectivity loss.
  • Anionic charge loss may stem from heparan sulfate proteoglycan defects or neutralizing cationic proteins.
  • Recurrence post-transplant suggests a humoral factor influencing glomerular permeability.

Purpose of the Study:

  • To review the pathophysiology and current treatment strategies for idiopathic nephrotic syndrome in children.
  • To evaluate the efficacy and limitations of various therapeutic agents.
  • To highlight challenges in managing steroid-resistant and steroid-dependent cases.

Main Methods:

  • Literature review of studies on idiopathic nephrotic syndrome.
  • Analysis of treatment outcomes with corticosteroids, levamisole, cyclosporine, and alkylating agents.
  • Discussion of pathomechanisms including glomerular charge defects and humoral factors.

Main Results:

  • Corticosteroids are the mainstay, with most patients responding but frequently relapsing.
  • Levamisole may prevent relapses; cyclosporine is effective in steroid-dependent cases but relapses occur upon cessation.
  • Alkylating agents show limited benefit in steroid-resistant INS; cyclosporine with prednisone increases nephrotoxicity risk.

Conclusions:

  • Corticosteroids remain fundamental for INS, despite high relapse rates.
  • Cyclosporine offers a valuable option for steroid-dependent or resistant cases, though long-term efficacy is limited by relapse upon withdrawal.
  • Further research is needed for optimal management of steroid-resistant INS and minimizing treatment-related toxicities.

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