Histopathological spectrum of childhood idiopathic steroid-resistant nephrotic syndrome in Tunisia

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Tahar Gargah
Aymen Labessi
Rim Goucha-Louzir
Fatma Ben Moussa
Mohamed Rachid Lakhoua

Abstract

Background: In children, renal biopsy is routinely required in the management of idiopathic steroid-resistant nephrotic syndrome particularly prior to starting nephrotoxic immunosuppressive agents.
Aim: To investigate the correlations between the results of initial renal biopsy in Tunisian children with idiopathic steroid-resistant nephrotic syndrome and the subsequent response to cyclosporineprednisolone combination.
Methods: We conducted a retrospective study of children with idiopathic steroid-resistant nephrotic syndrome over the period 2002- 2009. Data on clinico-biological features, histological diagnosis and response to cyclosporine-prednisolone were collected.
Results: Thirty patients were enrolled, of whom 16 had focal segmental glomerulosclerosis, eight had minimal change disease and six had diffuse mesangial proliferation. Complete Remission was achieved in 15 patients (50%). Nine patients (30%) went into partial remission. Only six patients presented no response (20%). No statistically significant relationship between the different pathological types and the response to CsA-prednisone was found.
Conclusion: In our study, two important facts were noted: 1) the predominant histopathological subtype was the focal segmental glomerulosclerosis; 2) a high remission rate was achieved in our patients using a combined cyclosporine-prednisolone treatment regimen. This response is not dependent on the histological type.

Keywords:

Children, Nephrotic syndrome, Steroid-resistance, Renal biopsy, Focal segmental glomerulosclerosis;, Cyclosporine

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References

  1. Borges FF, Shiraichi L, Da Silva MP, Nishimoto EI, Nougueira PC. Is focal segmental glomerulosclrrosis increasing in patients with nephrotic syndrome? Pediatr Nephrol 2007;22:1309-13.
  2. Kirpekar R, Yorgin PD, Tune BM, Kim MK, Sibley RK. Clinicopathologic correlates predict the outcome in children with steroid-resistant idiopathic nephrotic syndrome treated with pulse methylprednisolone therapy. Am J Kidney Dis 2002; 39:1143-52.
  3. Niaudet P. Treatment of childhood steroid-resistant idiopathic nephrosis with a combination of cyclosporine and prednisone. J Pediatr 1994;126: 981-6.
  4. Tarshish P, Tobin JN, Bernstein J, Edelmann CM. Prognostic significance of the early course of minimal change nephrotic syndrome: report of the International Study of Kidney. J Am Soc Nephrol 1997;8: 769-76.
  5. Kim JS, Bellew CA, Silverstein DM, Aviles DH, Boineau FG, Vehaskri VM. High incidence of initial and late resistance in childhood nephrotic syndrome. Kidney Int 2005;68: 1275-81.
  6. Bagga A, Mantan M. Nephrotic syndrome in children. Indian J Med Res 2005;122:13-28.
  7. Indian Society of Pediatric nephrology. Management of steroid resistant nephrotic syndrome. Indian Pediatr 2009;17:35-47.
  8. Fujinaga S, Kaneko K, Muto T, Ohtomo H, Murakami H, Yamashiro Y. Independent risk factors for chronic cyclosporine induced nephropathy in children with nephrotic syndrome. Arch Dis Child 2006; 91: 666-70.
  9. Brodehl J. The treatment of minimal change nephrotic syndrome: lessons learned from multicentre co-operative studies. Eur J Pediatr 1991; 150:380-7.
  10. Nammalwar BR, Vijayakumar M, Prahlad N. Experience of renal biopsy in children with nephrotic syndrome. Pediatr Nephrol 2006;21: 286-8.
  11. Gulati S, Sengupta D, Sharma RK, Sharma A, Gupta RK, Sing U, Gupta A. Steroid resistant nephrotic syndrome: role of histopathology. Indian Pediatr 2006;43:55-60.
  12. Kari J A, Halawani M, Mokhtar G, Jalalah SM, Anshasi W. Histopathology of steroid-resistant nephrotic syndrome in children living in the Kingdom of Saudi Arabia. Pediatr Nephrol 2009;24:1429-30.
  13. Hamasaki Y, Yoshikawa N, Hattori S, Sasaki S, Iijima K, Nakanishi K, et al. Cyclosporine and steroid therapy in children with steroid-resistant nephrotic syndrome. Pediatr Nephrol 2009;24:2177-85.
  14. Mekahli D, Liutkus A, Ranchin B, et al. Long-term outcome of idiopathic steroid-resistant nephrotic syndrome: a multicenter study. Pediatr Nephrol 2009; 24:1525-32.
  15. Thomas DB, Franceschini N, Hogan SL, et al. Clinical and pathological characteristics of focal segmental glomerulosclerosis pathologic variants. Kidney Int 2006; 69: 920-26.
  16. Habashy D, Hodson EM, Craig JC. Interventions for steroidresistant nephrotic syndrome: a systematic review. Pediatr Nephrol 2003; 18:906-12.
  17. Cattran DC, Alexopoulos E, Heering P, et al. Cyclosporin in idiopathic glomerular disease associated with the nephrotic syndrome: workshop recommendations. Kidney Int 2007;72:1429- 47.
  18. International Study of Kidney Disease in Children. Primary nephrotic syndrome in children: clinical significance of histopathologic variants of minimal change and of diffuse mesangial hypercellularity. Kidney Int 1981; 20: 765-71
  19. Hymes LC. Steroid-resistant, cyclosporine-responsive, relapsing nephrotic syndrome. Pediatr Nephrol 1995;9:137-9.
  20. Fuentes GM, Mesequer CG, carrion AP, Garcia-Pose A, Torres MN. Long-term outcome of focal segmental glomerulosclerosis after pediatric renal transplantation. Pediatr Nephrol 2010;25:529-34.