Clinical course and outcome of children with steroid-sensitive nephrotic syndrome

Pediatr Nephrol. 2011 Jul;26(7):1089-93. doi: 10.1007/s00467-011-1837-6. Epub 2011 Mar 12.

Abstract

We conducted a retrospective study on children with primary nephrotic syndrome (NS) to evaluate the clinical course and outcome of children with steroid-sensitive NS (SSNS). The medical records of 226 children, median 3.46 years (min 1.00, max 15.08) who referred to our clinics with SSNS between January 1978 and September 2005 were reviewed and entered into the study. Minimum duration of follow-up was 5 years and maximum 20 years (median 7.25 years). Of 226 patients who were treated with corticosteroids, 38 (16.8%) had no relapse but the remaining 188 (83.2%) patients experienced several relapses of which 128 patients (56.6%) required additional immunosuppressive agents for the remission. Of these, 122 (95%) were treated with levamisole, 22 (17%) with cyclosporine, 36 (28%) with cyclophosphamide, and ten (7.8 %) treated with mycophenolate mofetil. Several patients had to switch from one medication to others due to lack of response. On the last follow-up visit, 64(28.3%) patients were still under treatment, some patients had taken all of the above-mentioned drugs but still had multiple recurrences. Only 103 (45.5%) patients were in remission off the drug more than 3 years. This study shows that nearly one-third of pediatric patients with SSNS experience frequent relapses despite the combination of multiple immunosuppressive medications, which may continue until adulthood.

MeSH terms

  • Adolescent
  • Adrenal Cortex Hormones / therapeutic use*
  • Biopsy
  • Child
  • Child, Preschool
  • Drug Substitution
  • Drug Therapy, Combination
  • Female
  • Humans
  • Immunosuppressive Agents / therapeutic use*
  • Infant
  • Iran
  • Male
  • Nephrotic Syndrome / diagnosis
  • Nephrotic Syndrome / drug therapy*
  • Recurrence
  • Retrospective Studies
  • Time Factors
  • Treatment Outcome

Substances

  • Adrenal Cortex Hormones
  • Immunosuppressive Agents