title: Primary focal segmental glomerulosclerosis in Egyptian children: a 10-year single-centre experience.
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.
authors.affiliation: Pediatric Nephrology Unit, Mansoura University Children's Hospital, Mansoura University, Algomhoria Street, Mansoura, Egypt. Arefaey72@mans.edu.eg
authors.forename: Ahmed M
authors.initials: AM
authors.lastname: El-Refaey
authors.forename: Ashraf
authors.initials: A
authors.lastname: Bakr
authors.forename: Ayman
authors.initials: A
authors.lastname: Hammad
authors.forename: Atef
authors.initials: A
authors.lastname: Elmougy
authors.forename: Fatma
authors.initials: F
authors.lastname: El-Houseeny
authors.forename: Ashraf
authors.initials: A
authors.lastname: Abdelrahman
authors.forename: Amr
authors.initials: A
authors.lastname: Sarhan
mesh_terms: D000293:Adolescent; D001706:Biopsy; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D015897:Comorbidity; D016572:Cyclosporine / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D004534:Egypt / Q000453:epidemiology; D005260:Female; D005923:Glomerulosclerosis, Focal Segmental / Q000188:drug therapy / Q000453:epidemiology* / Q000473:pathology; D006417:Hematuria / Q000188:drug therapy / Q000453:epidemiology* / Q000209:etiology; D006801:Humans; D006973:Hypertension / Q000453:epidemiology* / Q000209:etiology / Q000473:pathology; D007223:Infant; D007676:Kidney Failure, Chronic / Q000453:epidemiology* / Q000473:pathology; D008297:Male; D008775:Methylprednisolone / Q000627:therapeutic use; D051436:Renal Insufficiency, Chronic / Q000188:drug therapy / Q000453:epidemiology* / Q000473:pathology; D012189:Retrospective Studies
chemical_list: D016572:Cyclosporine; D008775:Methylprednisolone