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Primary focal segmental glomerulosclerosis in Egyptian children: a 10-year single-centre experience

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Abstract

Primary focal segmental glomerulosclerosis (FSGS) in children accounts for 7–20% of all cases of idiopathic nephrotic syndrome (NS). To evaluate the clinical course of children with primary FSGS, the records of 72 patients with biopsy-proven FSGS admitted between 1995 and 2008 were retrospectively analysed. Forty-eight patients were male (66.7%). The mean age at presentation was 76.5 ± 54 (range 12–192) months. The mean duration of follow-up was 76.3 ± 42 (range 9–156) months. Seventeen patients (23.6%) presented with gross haematuria at initial presentation, 15 (20.8%) presented with hypertension and 10 patients (14%) presented with chronic renal insufficiency (CRI). The initial response to oral prednisolone was steroid resistance in 63 patients (87.5%) and steroid dependence in 4 patients (5%), while 5 patients (6.9%) were biopsied from the start because of atypical presentation. According to pathological classification, there were the tip variant (2%), collapsing variant (6%), perihilar variant (7%) and NOS (not otherwise specified; 85%). At the last clinical visit, 12 patients (16.7%) were in complete remission and 11 (15.3%) had progressed to CRI. Renal survival rates at 5 and 10 years were 93% and 68%, respectively. In conclusion, in this study we had a low percentage of FSGS, and fewer patients presenting with gross haematuria and hypertension compared with previous reports. In addition, the short-term overall renal survival seems to be better in our cohort.

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Acknowledgement

We are grateful to K.S. Adediran for his efforts in revision and reworking of the manuscript.

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Authors and Affiliations

  1. Pediatric Nephrology Unit, Mansoura University Children’s Hospital, Mansoura University, Algomhoria Street, Mansoura, Egypt

    Ahmed M. El-Refaey, Ashraf Bakr, Ayman Hammad, Atef Elmougy & Amr Sarhan

  2. Department of Pathology, Mansoura University Children’s Hospital, Mansoura University, Mansoura, Egypt

    Fatma El-Houseeny

  3. Department of Radiology, Mansoura University Children’s Hospital, Mansoura University, Mansoura, Egypt

    Ashraf Abdelrahman

Authors
  1. Ahmed M. El-Refaey
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  2. Ashraf Bakr
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  3. Ayman Hammad
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  4. Atef Elmougy
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  5. Fatma El-Houseeny
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  6. Ashraf Abdelrahman
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Correspondence to Ahmed M. El-Refaey.

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El-Refaey, A.M., Bakr, A., Hammad, A. et al. Primary focal segmental glomerulosclerosis in Egyptian children: a 10-year single-centre experience. Pediatr Nephrol 25, 1369–1373 (2010). https://doi.org/10.1007/s00467-010-1448-7

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  • DOI: https://doi.org/10.1007/s00467-010-1448-7

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