Focal segmental glomerulosclerosis in children

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Published Sep 9, 2016
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Vol. 94 No. 5 (2016): Issue May 2016

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Manel Jellouli
Kamel Abidi
Mouna Askri
Meriem Ferjani
Ouns Naija
Yousra Hammi
Rym Goucha
Tahar Gargah

Abstract

Abstract:
Background: Focal segmental glomerulosclerosis (FSGS) represents 20% of nephrotic syndrome in children. The clinical course and prognosis is heterogeneous in children. The aim of this study was to analyze treatment and outcome of children with FSGS.
Methods: This retrospective study was conducted in the Department of Pediatrics in Charles Nicolle Hospital during a 15-year period (1996-2010).
Results: There were 30 children, 16 boys and 14 girls. The mean age was 7 ± 4 years. Nephrotic syndrome was observed in 26 patients, hematuria was noticed in 2 patients and renal insufficiency was detected in 2 patients at presentation. FSGS, not otherwise specified, was the predominant variant. All patients with nephrotic syndrome were treated with steroids. Only three patients responded to it. Twenty one patients were treated with cisclosporin A and this resulted in a 57% complete remission and a 24% partial response. Cyclophosphamide was administered to 6 patients and engendered a 50% complete remission. Six patients were treated with mycophenolate mophetil and showed no response in all cases. Renal insufficiency has been developed in 12 children.
Conclusion: Results from this study showed that the majority of children with FSGS achieve a high sustained remission rate with ciclosporine A.

Keywords:

Children, focal segmental glomerulosclerosis, renal insufficiency, nephrotic syndrome.

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