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Glomerular Outgrowth as an Ex Vivo Assay to Analyze Pathways Involved in Parietal Epithelial Cell Activation
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Primary focal segmental glomerulosclerosis in Egyptian children: a 10-year single-centre experience

Ahmed M El-Refaey1, Ashraf Bakr, Ayman Hammad

  • 1Pediatric Nephrology Unit, Mansoura University Children's Hospital, Mansoura University, Algomhoria Street, Mansoura, Egypt. Arefaey72@mans.edu.eg

Pediatric Nephrology (Berlin, Germany)
|March 3, 2010
PubMed

Insights

This study analyzed 72 children with primary focal segmental glomerulosclerosis (FSGS), finding a low incidence and better short-term renal survival compared to previous reports. Most children showed steroid resistance, with significant progression to chronic renal insufficiency (CRI).

Area of Science:

  • Pediatric Nephrology
  • Glomerular Diseases
  • Renal Pathology

Background:

  • Primary focal segmental glomerulosclerosis (FSGS) is a significant cause of nephrotic syndrome (NS) in children.
  • Understanding the clinical course and outcomes of pediatric FSGS is crucial for management.

Purpose of the Study:

  • To evaluate the clinical course and outcomes of children diagnosed with primary FSGS.
  • To assess renal survival rates in a cohort of pediatric FSGS patients.

Main Methods:

  • Retrospective analysis of 72 children with biopsy-proven FSGS between 1995 and 2008.
  • Evaluation of clinical presentation, response to treatment, pathological variants, and long-term renal survival.

Main Results:

  • Most patients (87.5%) exhibited steroid resistance to oral prednisolone.
  • 16.7% achieved complete remission, while 15.3% progressed to chronic renal insufficiency (CRI).
  • Five and 10-year renal survival rates were 93% and 68%, respectively, with a lower incidence of gross hematuria and hypertension than previously reported.

Conclusions:

  • The study observed a lower percentage of FSGS and fewer cases with gross hematuria and hypertension compared to prior studies.
  • Short-term renal survival in this pediatric FSGS cohort appears to be favorable.

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