Management and Outcome of Steroid-Resistant Nephrotic Syndrome in Children

Authors

  • Hassan Otukesh Department of Nephrology, Ali Asghar Children Hospital, Iran University of Medical Sciences, Tehran, Iran Author
  • Salman Otukesh Ali Asghar Children Hospital, Iran University of Medical Sciences, Tehran, Iran Author
  • Mona Mojtahedzadeh Ali Asghar Children Hospital, Iran University of Medical Sciences, Tehran, Iran Author
  • Rozita Hoseini Department of Nephrology, Ali Asghar Children Hospital, Iran University of Medical Sciences, Tehran, Iran Author
  • Seyed-Mohammad Fereshtehnejad Ali Asghar Children Hospital, Iran University of Medical Sciences, Tehran, Iran Author
  • Azam Riahi Fard Iran University of Medical Sciences and Health Services, Tehran, Iran Author
  • Nader Sadigh Ali Asghar Children Hospital, Iran University of Medical Sciences, Tehran, Iran Author
  • Ashkan Heshmatzade Behzadi Ali Asghar Children Hospital, Iran University of Medical Sciences, Tehran, Iran Author
  • Ramila Javadi Ali Asghar Children Hospital, Iran University of Medical Sciences, Tehran, Iran Author
  • Nakissa Hooman Department of Nephrology, Ali Asghar Children Hospital, Iran University of Medical Sciences, Tehran, Iran Author
  • Mitra Mehrazma Department of Pathology, Iran University of Medical Sciences, Tehran, Iran Author

Abstract

Introduction. Steroid-resistant nephrotic syndrome (SRNS) is uncommon in children, but often leads to ESRD. We report our experience with SRNS and its treatments and outcomes.

Materials and Methods. We assessed 73 children with SRNS admitted to Ali Asghar Children Hospital in Tehran, Iran. Their clinical presentations, treatment, and disease courses were reviewed. The mean follow-up duration was 6.0 ± 4.2 years. Moreover, survival times were calculated and the Cox regression method was used to determine variables able to predict survival of the kidneys.

Results. Age at the onset of the disease, sex, and hematuria were not predictive of the response to treatment with immunosuppressive drugs in the children with SRNS. The type of resistance (early or late) was associated with the responsiveness to immunosuppressives. Response to any of the immunosuppressive drugs determined the responsiveness to other immunosuppressive drugs. Cyclosporine was more effective than cyclophosphamide as initial therapy. The mean kidney survival time was 11.62 years. Kidney survival rates were 94.6%, 70.0%, 56.0%, and 34.0% at 1, 5, 10, and 15 years, respectively, in patients with initial resistance to steroid, while these were 100%, 100%, 83.0%, and 83.0% in those with late resistance, respectively (P = .03).

Conclusions. We showed that patients with late steroid resistance had better response to immunosuprressive drugs than patients with early resistance. We also showed that resistance to immunosuppressive therapies increased the risk of resistance to other immunosuppressive drugs. Achievement of complete or partial remission with any therapy reduced the risk of ESRD.

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Published

2009-10-12

Issue

Section

ORIGINAL | Kidney Diseases

How to Cite

Management and Outcome of Steroid-Resistant Nephrotic Syndrome in Children. (2009). Iranian Journal of Kidney Diseases, 3(4), 210-217. https://www.ijkd.org/index.php/ijkd/article/view/122

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