For citation purposes: Naushabayeva A, Abeuova B, Chingayeva G, Kabulbayev K, Nurbekova A, Nugmanova A, Smagulova A, Kanatbayeva A. Focal segmental glomerulosclerosis variants in children with nephrotic syndrome. OA Nephrology 2013 Sep 01;1(2):16.

Research study

 
Treatment

Focal segmental glomerulosclerosis variants in children with nephrotic syndrome

A Naushabayeva, B Abeuova, G Chingayeva, K Kabulbayev, A Nurbekova, A Nugmanova, A Smagulova, A Kanatbayeva
 

Authors affiliations

(1) Nephrology Department, The Kazakh National Medical University named after S.D. Asfendiyarov, Almaty, Kazakhstan

(2) Pediatrics Department, Karaganda State Medical University, Karaganda, Kazakhstan

*Corresponding author Email: assiya.naushabayeva@gmail.com

Abstract

Introduction

The aim of this study was to determine pathological variants of childhood focal segmental glomerulosclerosis and assess efficacy of prednisolone + methylprednisolone + cyclosporin A treatment.

Materials and methods

This retrospective cohort study included 134 native Kazakh children (3 months–17 years) with nephrotic syndrome hospitalised in the Nephrology Department of the Republic Children's Clinical Hospital from 2004 to 2011. Kidney biopsy and pathological investigations were performed in 38 nephrotic syndrome patients with proven steroid resistance.

Results

Focal segmental glomerulosclerosis was confirmed in 38 (28.4%) patients (treatment group). The main focal segmental glomerulosclerosis variants were glomerular tip lesions and not otherwise specified types. Historical (did not undergo kidney biopsy) controls were treated with cyclosporin A or alkylating agents. Prednisolone + methylprednisolone + cyclosporin A immunosuppressive therapy was highly effective, allowing complete remission in 88.9% patients with minimum side effects. Patients with the not otherwise specified variant (NPHS2 and WT1 mutations) did not achieve remission. Combination cyclosporin A treatment was significantly more effective than alkylating agent treatment in steroid-resistant nephrotic syndrome patients without genetic mutations.

Conclusion

Tip lesions are predominant in childhood steroid-resistant nephrotic syndrome. Prednisolone + methylprednisolone + cyclosporin A therapy is safe and effective for childhood focal segmental glomerulosclerosis. Establishing a podocyte mutation profile is important to predict treatment outcome.

Licensee OA Publishing London 2013. Creative Commons Attribution License (CC-BY)
Keywords