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Management and outcome of steroid-resistant nephrotic syndrome in children

Hassan Otukesh1, Salman Otukesh, Mona Mojtahedzadeh

  • 1Department of Nephrology, Ali Asghar Children Hospital, Iran University of Medical Sciences, Tehran, Iran. hasanotukesh@yahoo.com

Iranian Journal of Kidney Diseases
|October 21, 2009
PubMed

Insights

Children with late steroid-resistant nephrotic syndrome (SRNS) respond better to immunosuppressive drugs. Achieving remission reduces the risk of end-stage renal disease (ESRD).

Area of Science:

  • Pediatric Nephrology
  • Immunosuppressive Therapy
  • Kidney Disease Outcomes

Background:

  • Steroid-resistant nephrotic syndrome (SRNS) is uncommon in children but frequently progresses to end-stage renal disease (ESRD).
  • Understanding treatment responses and outcomes in pediatric SRNS is crucial for improving patient prognosis.

Purpose of the Study:

  • To evaluate the treatment responses and long-term outcomes of pediatric patients with SRNS.
  • To identify factors predicting kidney survival in children with SRNS.

Main Methods:

  • Retrospective review of 73 children with SRNS treated at Ali Asghar Children Hospital.
  • Analysis of clinical presentations, treatment regimens, disease courses, and kidney survival.
  • Utilized Cox regression to identify predictors of kidney survival.

Main Results:

  • Late steroid resistance predicted a better response to immunosuppressive drugs compared to early resistance (P = .03).
  • Initial therapy with cyclosporine was more effective than cyclophosphamide.
  • Kidney survival rates at 15 years were 34% for early resistance and 83% for late resistance.
  • Achieving remission with any therapy significantly reduced the risk of ESRD.

Conclusions:

  • Late steroid resistance in pediatric SRNS is associated with a more favorable response to immunosuppressive therapies.
  • Resistance to one immunosuppressive drug increases the likelihood of resistance to others.
  • Early and complete remission is key to preventing progression to ESRD in children with SRNS.
Abstract

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