Preview

Current Pediatrics

Advanced search

TREATMENT OF CHILDREN WITH FOCAL SEGMENTAL GLOMERULAR SCLEROSIS WITH CYCLOSPORINE A

Abstract

The article demonstrates successful experience of treatment of focal segmental glomerular sclerosis (FSGS) with cyclosporine A in children. 25 children over the age 1,5–16 years old with FSGS were treated with cyclosporine A in medium dose 4–5 mg/kg combined with prednisolone 1–1,5 mg/kg every other day. Pulse treatment with methylprednisolone 30 mg/kg every other day, in total 3–9 injections, was administrated for the purpose of remission induction. After 5 months of treatment with cyclosporine A complete clinical and laboratory remission of steroid-resistance nephritic syndrome (SRNS) was achieved in 9 (36%) patients, partial response was registered in 6 (24%) patients, maintenance of SRNS activity was detected in 10 (40%) cases. After the year of treatment complete remission was shown in 13 (52%) children, partial response — in 5 (20%) patients, and absence of effect was registered in 7 (28%) cases. In 56% of cases, the treatment with steroids was completely stopped after achievement of SRNS remission within administration of cyclosporine A. At the present times, authors observe 14 children, whose duration of treatment with cyclosporine A is 2 years. 12 patients remain previously achieved complete or partial remission of nephritic syndrome without decrease of nitrogen excretion function of kidneys. 9 patients underwent repeated biopsy of kidneys for the exclusion of nephrotoxic effect of cyclosporine A. The results of biopsy allowed prolongation of therapy: in 6 children the previous dose maintained, and 3 children got double decreased dose. Thus, the treatment with cyclosporine A is effective in 76% of patients with FSGS. Prolongation of treatment with cyclosporine A up to 2 years and more is possible in conditions of regular diagnostic of kidneys function and absence of its nephrotoxic effect signs, according to a data from repeated biopsy.
Key words: children, focal segmental glomerular sclerosis, cyclosporine, methylprednisolone.
(Voprosy sovremennoi pediatrii — Current Pediatrics. 2009;8(5):93-97)

About the Authors

O.V. Komarova
Scientific Center of Children’s Health, Russian Academy of Medical Sciences, Moscow
Russian Federation


A.N. Tsygin
Scientific Center of Children’s Health, Russian Academy of Medical Sciences, Moscow
Russian Federation


L.V. Leonova
Scientific Center of Children’s Health, Russian Academy of Medical Sciences, Moscow
Russian Federation


A.G. Kucherenko
Scientific Center of Children’s Health, Russian Academy of Medical Sciences, Moscow
Russian Federation


O.A. Zrobok
Scientific Center of Children’s Health, Russian Academy of Medical Sciences, Moscow
Russian Federation


T.V. Vashurina
Scientific Center of Children’s Health, Russian Academy of Medical Sciences, Moscow
Russian Federation


M.V. Rozhkova
Scientific Center of Children’s Health, Russian Academy of Medical Sciences, Moscow
Russian Federation


References

1. Haas M., Meehan S.M., Karrison T.G., Spargo B.H. Changing etiologies of unexplained adult nephrotic syndrome: a comparison of renal biopsy findings from 1976–1979 and 1995–1997. Am. J. Kidney Dis. 1997; 30 (5): 621–631.

2. Braden G.L., Mulhern J.G., O'Shea M.H. et al. Changing incidence of glomerular diseases in adults. Am. J. Kidney Dis. 2000; 35 (5): 878–883.

3. Ichikawa I., Fogo A. Focal segmental glomerulosclerosis. Pediatr. Nephrol. 2004; 10 (1): 347–391.

4. Korbet S.M. Treatment of primary focal segmental glomerulosclerosis. J. Am. Soc. Nephrol. 2002; 62: 2301–2310.

5. Lieberman K.V., Tejani A.A. randomized double blind placebo controlled trial of cyclosporine in steroid-resistant focal segmental glomerulosclerosis in children. J. Am. Soc. Nephrol. 1996; 7 (1): 56–63.

6. Tsygin A., Sergeeva T., Voznesenskaya T. et al. Growing incidense of focal and segmental glomerulosclerosis as a cause of steroid resistent nephrotic syndrome in Russian children. Paediatric clinical nephrology, acute and chronic renal failure (Abstract E2). 2004. P. 259.

7. Rydel J.J., Korbet S.M., Borok R.Z., Schwartz M.M. Focal segmental glomerular sclerosis in adults: presentation, course, and response to treatment. Am. J. Kidney Dis. 1995; 25 (4): 534–542.

8. Tune B.M., Mendoza S.A. Treatment of the idiopathic nephrotic syndrome: regimens and outcomes in children and adults. J. Am. Soc. Nephrol. 2001; 12: 44–47.

9. Цыгин А.Н., Комарова О.В., Сергеева Т.В. и др. Нефротический синдром. Клинические рекомендации по педиатрии. Под ред. А.А. Баранова. М.: Гэотар-Медиа. 2005. C. 107–128.

10. Suthanthiran M., Strom T.B. Immunoregulatory drugs: mechanistic basis for use in organ transplantation. Pediatr. Nephrol. 1997; 11 (5): 651–657.

11. Scheinman R.I., Cogswell P.G., Lofquist A.K., Baldwin A.S. Role of transcription activation of Iβα in mediation of immunosupression by glucocorticoids. Sience. 1995; 270: 283–286.

12. Meyrier A., Simon P., Perret G., Condamin-Meyrier M.C. Remission of idiopathic nephrotic syndrome after treatment with cyclosporine. Br. Med. J. 1986; 292 (6523): 789–792.

13. Tejani A., Butt K., Trachtman H. et al. Cyclosporine A induced remission of relapsing nephrotic syndrome in children. Kidney Int. 1988; 33: 729–734.

14. Klein M., Radhakrishnan J., Appel G. Cyclosporine treatment of glomerular diseases. Ann. Rev. Med. 1999; 50: 1–15.

15. Kveder R. Therapy-resistant focal and segmental glomerulosclerosis. Nephrol. Dial. Transplant. 2003; 18: 34–37.

16. Korbet S.M., Schwartz M.M., Lewis E.J. Primary focal segmental glomerulosclerosis: clinical course and response to therapy. Am. J. Kidney Dis. 1994; 23 (6): 773–783.

17. Korbet S.M. Angiotensin antagonists and steroids in the treatment of focal segmental glomerulosclerosis. Semin. Nephrol. 2003; 23 (2): 219–228.

18. Niaudet P., for the French Society of Pediatric Nephrology. Treatment of childhood steroid resistant idiopathic nephrosis with a combination of cyclosporine and prednisone. J. Pediatr. 1994; 125 (5): 981–986.

19. Waldo F.B., Benfield M.R., Kohaut E.C. Therapy of focal and segmental glomerulosclerosis with methylprednisolone, cyclosporine A, and prednisone. Pediatr. Nephrol. 2009; 12 (5): 397–400.

20. Cattran D.C., Appel G.B., Hebert L.A. et al. A randomized trial of cyclosporine in patients with steroid-resistant focal segmental glomerulosclerosis. Kidney Int. 1999; 56 (6): 2220–2226.

21. Ponticelli C., Rizzoni G., Edefonti A. et al. A randomized trial of cyclosporine in steroid-resistant idiopathic nephrotic syndrome. Kidney Int. 1993; 43 (6): 1377–1384.

22. Burgess E. Management of focal segmental glomerulosclerosis: evidence-based recommendations. Kidney Int. Suppl. 1999; 70: 26–32.

23. El-Husseini A., El-Basuony F., Mahmoud I. et al. Long-term effects of cyclosporine in children with idiopathic nephrotic syndrome: a single centre experience. Nephrol. Dial. Transplant. 2005; 20 (11): 2433–2438.

24. Meyrier A., Noel L.H., Auriche P., Callard P. Long-term renal tolerance of cyclosporin A treatment in adult idiopathic nephrotic syndrome. Collaborative Group of the Societe de Nephrologie. Kidney Int. 1994; 45 (5): 1446–1456.

25. Ehrich J.H., Geerlings C., Zivicnjak M. et al. Steroid-resistant idiopathic childhood nephrosis: overdiagnosed and undertreated. Nephrol. Dial. Transplant. 2007; 22 (8): 2183-2193.

26. Sümegi V., Haszon I., Bereczki C. et al. Long term follow up after cyclophosphamide and cyclosporine-A therapy in steroid dependent and resistant nephrotic syndrome. Pediatr. Nephrol. 2008; 23 (7): 1085–1092.


Review

For citations:


Komarova O., Tsygin A., Leonova L., Kucherenko A., Zrobok O., Vashurina T., Rozhkova M. TREATMENT OF CHILDREN WITH FOCAL SEGMENTAL GLOMERULAR SCLEROSIS WITH CYCLOSPORINE A. Current Pediatrics. 2009;8(5):93-97.

Views: 503


ISSN 1682-5527 (Print)
ISSN 1682-5535 (Online)