Document Detail


Primary focal segmental glomerulosclerosis in Egyptian children: a 10-year single-centre experience.
MedLine Citation:
PMID:  20195645     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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:
Ahmed M El-Refaey; Ashraf Bakr; Ayman Hammad; Atef Elmougy; Fatma El-Houseeny; Ashraf Abdelrahman; Amr Sarhan
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Publication Detail:
Type:  Journal Article     Date:  2010-03-02
Journal Detail:
Title:  Pediatric nephrology (Berlin, Germany)     Volume:  25     ISSN:  1432-198X     ISO Abbreviation:  Pediatr. Nephrol.     Publication Date:  2010 Jul 
Date Detail:
Created Date:  2010-05-21     Completed Date:  2010-08-26     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8708728     Medline TA:  Pediatr Nephrol     Country:  Germany    
Other Details:
Languages:  eng     Pagination:  1369-73     Citation Subset:  IM    
Affiliation:
Pediatric Nephrology Unit, Mansoura University Children's Hospital, Mansoura University, Algomhoria Street, Mansoura, Egypt. Arefaey72@mans.edu.eg
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Biopsy
Child
Child, Preschool
Cohort Studies
Comorbidity
Cyclosporine / therapeutic use
Drug Therapy, Combination
Egypt / epidemiology
Female
Glomerulosclerosis, Focal Segmental / drug therapy,  epidemiology*,  pathology
Hematuria / drug therapy,  epidemiology*,  etiology
Humans
Hypertension / epidemiology*,  etiology,  pathology
Infant
Kidney Failure, Chronic / epidemiology*,  pathology
Male
Methylprednisolone / therapeutic use
Renal Insufficiency, Chronic / drug therapy,  epidemiology*,  pathology
Retrospective Studies
Chemical
Reg. No./Substance:
59865-13-3/Cyclosporine; 83-43-2/Methylprednisolone

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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