European Journal of Pediatrics

, Volume 151, Issue 10, pp 775–778 | Cite as

Long-term low-dose cyclosporin A in steroid dependent nephrotic syndrome of childhood

  • T. J. Neuhaus
  • H. R. Burger
  • M. Klingler
  • A. Fanconi
  • E. P. Leumann


Therapy of steroid-dependent idiopathic nephrotic syndrome is often unsatisfactory. Since 1986 we have treated nine children (six male and three female), aged 3–16 years, with cyclosporin A (CsA) during 2.0–5.2 (median 3.1) years. All had minimal change disease on renal biopsy and had previously received cyclophosphamide. Mean daily dosage of CsA was 4.1 mg/kg (range 2.7–5.8) and mean whole blood trough level was 220ng/ml (range 141–271). The relapse rate decreased from 3.4/patient year before CsA to 0.55 on CsA. Discontinuation of CsA or reduction below 2 mg/kg daily was always followed by a relapse. The overall relapse rate, including the period with very low-dose CsA, was 0.95/patient year. Four patients required additional low-dose alternate-day prednisone. Repeat renal biopsy showed minimal change disease in eight patients and focal segmental glomerulosclerosis in one; CsA-toxicity was mild in two and moderate in one. The latter was the only patient with slightly reduced glomerular filtration rate. Two boys with delayed puberty spontaneously matured and reached expected final height. We conclude that long-term low-dose CsA is very effective and steroid-sparing. Its use is justified in selected patients, particularly in those with numerous relapses and in male patients before and during puberty, as long as renal function and CsA-toxicity are carefully monitored.

Key words

Nephrotic syndrome Steroid-dependent idiopathic nephrotic syndrome Cyclosporin A Children 



cyclosporin A




glomerular filtration rate


idiopathic nephrotic syndrome


Minimal Change disease


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. 1.
    Arbeitsgemeinschaft für pädiatrische Nephrologie (1981) Alternate-day prednisone is more effective than intermittent prednisone in frequently relapsing nephrotic syndrome. Eur J Pediatr 135:229–237Google Scholar
  2. 2.
    Arbeitsgemeinschaft für pädiatrische Nephrologie (1987) Cyclophosphamide treatment of steroid dependent nephrotic syndrome: comparison of eight week with 12 week course. Arch Dis Child 62:1102–1106Google Scholar
  3. 3.
    Arbeitsgemeinschaft für pädiatrische Nephrologie (1990) Cyclosporin A in MCNS: CsA Study II, (abstract). Pediatr Nephrol 4:C60Google Scholar
  4. 4.
    Borel JF (1991) Mechanism of action of cyclosporin A and rationale for its use in nephrotic syndrome. Clin Nephrol 35 [Suppl 1]:23–30Google Scholar
  5. 5.
    Brandis M, Burghard R, Leititis J, Zimmerhackl B, Hildebrandt F, Helmchen U (1988) Cyclosporine A for treatment of nephrotic syndromes. Transplant Proc 20 [Suppl 4]:275–279Google Scholar
  6. 6.
    Brodehl J, Hoyer PF (1985) Ciclosporin treatment of idiopathic nephrotic syndrome in children (minimal change disease and focal segmental glomerulosclerosis). In: Schindler R (ed) Ciclosporine in autoimmune diseases. Springer, Berlin Heidelberg New York, pp 329–333Google Scholar
  7. 7.
    Brodehl J, Brandis M, Helmchen U, Hoyer PF, Burghard R, Ehrlich JHH, Zimmerhackl RB, Klein W, Wonigeit K (1988) Cyclosporin A treatment in children with minimal change nephrotic syndrome and focal segmental glomerulosclerosis. Klin Wochenschr 66:1126–1137Google Scholar
  8. 8.
    Brodehl J, Hoyer PF, Oemar BS, Helmchen U, Wonigeit K (1988) Cyclosporine treatment of nephrotic syndrome in children. Transplant Proc 20 [Suppl 4]:269–274Google Scholar
  9. 9.
    Chantler C, Barratt TM (1972) Estimation of glomerular filtration rate from plasma clearance of 51 chronium edetic acid. Arch Dis Child 47:613–617Google Scholar
  10. 10.
    Collaborative Study Group of Sandimmun in Nephrotic Syndrome (1991) Safety and tolerability of cyclosporin A (Sandimmun) in idiopathic nephrotic syndrome. Clin Nephrol 35 [Suppl 1]:48–60Google Scholar
  11. 11.
    Feinstein EI, Chesney RW, Zelikovic I (1988) Peritonitis in childhood renal diseases Am J Nephrol 8:147–165Google Scholar
  12. 12.
    Giani M, Edefonti A, Ghio L, Longaretti A, Paterlini G, Tirelli S, Bettinelli A, Sereni F (1990) Treatment of idiopathic nephrotic syndrome with cyclosporine A: a control biopsy study (abstract). Pediatr Nephrol 4:C11Google Scholar
  13. 13.
    Gorensek MJ, Lebel MH, Nelson JD (1988) Peritonitis in children with nephrotic syndrome. Pediatrics 81:849–856Google Scholar
  14. 14.
    Hoyer PF, Offner G, Oemar BS, Brodehl J, Ringe B, Pichlmayr R (1990) Four years' experience with cyclosporin A in pediatric kidney transplantation. Acta Paediatr Scand 79:622–629Google Scholar
  15. 15.
    Hulton SA, Jadresic L, Risdon RA, Dillon MJ, Barratt TM (1991) Effect of cyclosporin A on renal function in childhood nephrotic syndrome (abstract). Pediatr Nephrol 5:C25Google Scholar
  16. 16.
    International Study of Kidney Disease in Children (1982) Early identification of frequent relapsers among children with minimal change nephrotic syndrome. J Pediatr 101:514–518Google Scholar
  17. 17.
    Kitano Y, Yoshikawa N, Tanaka R, Nakamura H, Ninomiya M, Ito H (1990) Ciclosporin treatment in children with steroid-dependent nephrotic syndrome. Pediatr Nephrol 4:474–477Google Scholar
  18. 18.
    Koskimies O, Vilska J, Rapola J, Hallman N (1982) Longterm outcome of primary nephrotic syndrome. Arch Dis Child 57:544–548Google Scholar
  19. 19.
    Lewis MA, Baildom EM, Davis N, Houston IB, Postlethwaite RJ (1989) Nephrotic syndrome: from toddlers to twenties. Lancet I:255–258Google Scholar
  20. 20.
    Mehls O, Andrassy K, Koderisch J, Herzog U, Ritz E (1987) Hemostasis and thromboembolism in children with nephrotic syndrome: differences from adults. J Pediatr 110:862–867Google Scholar
  21. 21.
    Melocoton TL, Kamil ES, Cohen AH, Fine RN (1991) Longterm cyclosporine-A treatment of steroid-resistant and steroid dependent nephrotic syndrome. Am J Kidney Dis 18:583–588Google Scholar
  22. 22.
    Meyrier A (1989) Treatment of glomerular diseases with cyclosporin A. Nephrol Dial Transplant 4:923–931Google Scholar
  23. 23.
    Meyrier A, Collaborative Group of the Societé de Nephrologie (1990) Long-term tolerance of cyclosporin A treatment in adult nephrosis (abstract). Nephrol Dial Transplant 5:652Google Scholar
  24. 24.
    Niaudet P, Habib R, Tete MJ, Hinglais N, Broyer M (1987) Cyclosporin in the treatment of idiopathic nephrotic syndrome in children. Pediatr Nephrol 1:566–573Google Scholar
  25. 25.
    Niaudet P, Broyer M, Habib R (1991) Treatment of idiopathic nephrotic syndrome with cyclosporin A in children. Clin Nephrol 35 [Suppl 1]:31–36Google Scholar
  26. 26.
    Offner G, Aschendorff C, Brodehl J (1991) Growth after renal transplantation: an update. Pediatr Nephrol 5:472–476Google Scholar
  27. 27.
    Ponticelli C, Rivolta E (1990) Ciclosporin in minimal change glomerulopathy and in focal segmental glomerular sclerosis. Am J Nephrol 10 [Suppl]:105–109Google Scholar
  28. 28.
    Rees L, Greene SA, Adlard P, Jones J, Haycock GB, Rigden SPA, Preece M, Chantler C (1988) Growth and endocrine function in steroid sensitive nephrotic syndrome. Arch Dis Child 63:484–490Google Scholar
  29. 29.
    Schnaper HW (1989) The immune system in minimal change nephrotic syndrome. Pediatr Nephrol 3:101–110Google Scholar
  30. 30.
    Soergel M, Altrogge H, Wingen A-M, Amon O, Müller-Wiefel DE, Schärer K (1991) Langzeitverlauf des nephrotischen Syndroms unter Ciclosporin A (abstract). Monatsschr Kinderheilkd 139:185Google Scholar
  31. 31.
    Tejani A, Butt K, Trachtmann H, Suthanthiran M, Rosenthal CJ, Khawar MR (1987) Cyclosporine-induced remission of relapsing nephrotic syndrome in children. J Pediatr 111:1056–1062Google Scholar
  32. 32.
    Trompeter RS, Lloyd BW, Hicks J, White RHR, Cameron JS (1985) Long-term outcome for children with minimal change nephrotic syndrome. Lancet I:255–258Google Scholar
  33. 33.
    Ueda N, Kuno K, Ito S (1990) Eight and 12 week courses of cyclophosphamide in nephrotic syndrome. Arch Dis Child 65:1147–1150Google Scholar

Copyright information

© Springer-Verlag 1992

Authors and Affiliations

  • T. J. Neuhaus
    • 1
  • H. R. Burger
    • 2
  • M. Klingler
    • 1
  • A. Fanconi
    • 1
  • E. P. Leumann
    • 1
  1. 1.University Children's HospitalZürichSwitzerland
  2. 2.Department of PathologyUniversity of ZurichZürichSwitzerland

Personalised recommendations