Prevalence and outcome of focal segmental glomerulosclerosis in Iranian children with nephrotic syndrome

Iran J Kidney Dis. 2012 Jan;6(1):18-24.

Abstract

Introduction: Some evidence suggest an increase in the prevalence of focal segmental glomerular sclerosis (FSGS) in children. To date, there has been no study of the outcome in children with FSGS and its frequency over several decades in Iran. We aimed to report the changing trend of FSGS incidence and its outcome in a sample of Iranian children.

Materials and methods: Between 1982 and 2008, all 716 kidney biopsies performed in children referred to Ali Asghar Children Hospital were recorded and confirmed cases with FSGS lesions were further evaluated. Baseline and clinical characteristics of all FSGS patients were assessed and the therapies and outcomes were reviewed.

Results: The incidence rate of FSGS was 10.1% between 1982 and 1990, which was significantly increased to as high as 20.5% after the year 2000 (P = .001). Among 64 children with FSGS, 20 progressed to end-stage renal disease with a mean survival time of 11.45 years (standard error of mean, 1.34 years). Kidney survival rates were 90.4%, 69%, and 47% at 1, 5 and 10 years of follow-up.

Conclusions: Our study demonstrates an increasing trend in FSGS incidence in Iranian children. However, kidney survival rates of our patients were similar to those reported by others in different countries.

MeSH terms

  • Child
  • Child, Preschool
  • Cyclophosphamide / therapeutic use
  • Cyclosporine / therapeutic use
  • Disease Progression
  • Female
  • Follow-Up Studies
  • Glomerulosclerosis, Focal Segmental / complications*
  • Glomerulosclerosis, Focal Segmental / drug therapy
  • Glomerulosclerosis, Focal Segmental / epidemiology*
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Incidence
  • Iran / epidemiology
  • Kaplan-Meier Estimate
  • Kidney Failure, Chronic / etiology
  • Male
  • Mycophenolic Acid / analogs & derivatives
  • Mycophenolic Acid / therapeutic use
  • Nephrotic Syndrome / complications*
  • Nephrotic Syndrome / drug therapy
  • Prevalence
  • Proportional Hazards Models
  • Steroids / therapeutic use
  • Treatment Outcome

Substances

  • Immunosuppressive Agents
  • Steroids
  • Cyclosporine
  • Cyclophosphamide
  • Mycophenolic Acid