Nephrology pp 1468-1473 | Cite as

New Therapies for the Treatment of Idiopathic Nephrotic Syndrome

  • Patrick Niaudet
  • Michel Broyer
  • Renée Habib


The treatment of children with idiopathic nephrotic syndrome who have multiple relapses or who are steroid-resistant is a difficult issue. Alkylating agents are often proposed but they have several side effects, including gonadal toxicity, which limit their use. Treatment with levamisole may be efficient in steroid-dependent patients, allowing prednisone to be tapered or stopped in approximately 50% of cases. Cyclosporine has proved to be effective in 85% of steroid-dependent patients, but most patients relapse when cyclosporine is tapered or withdrawn. Thus, in these patients, cyclosporine may be required for long periods of time. Conversely, cyclosporine alone is less effective in steroid-resistant patients, as only 28% of these patients respond to the treatment. Cyclosporine in association with prednisone may be a better alternative as, in our experience, 14 out of 31 patients went into remission with such treatment. It should be stressed that response to cyclosporine is better correlated with initial steroid responsiveness than with histological category. Eighteen out of 43 patients with serial renal biopsies developed significant tubulo interstitial lesions which could be attributable to cyclosporine nephrotoxicity. The risk of developing chronic nephrotoxicity appears to be higher in steroid-resistant patients. Cyclosporine nephrotoxicity is not related to the duration of treatment and may develop in patients with normal renal function.


Nephrotic Syndrome Idiopathic Nephrotic Syndrome Minimal Change Disease Cyclosporine Therapy Childhood Nephrotic Syndrome 
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  1. 1.
    Habib R, Churg J (1984) Minimal change disease, mesangial proliferation glomerulo-nephritis and focal sclerosis: individual entities or a spectrum. In: RR Robinson (ed) Nephrology. Springer, New York, pp 634–644CrossRefGoogle Scholar
  2. 2.
    Niaudet P, Habib R, Gagnadoux MF, Tête MJ, Broyer M (1988) Treatment of severe childhood nephrosis. In: Grunfeld JP, Bach JF, Crosnier J, Funk-Brentano JL, Maxwell MH (eds) Advances in nephrology. Year Book Medical, pp 151–172Google Scholar
  3. 3.
    Tanphaichitr P, Tanphaichitr D, Sureesatanan J (1980) Treatment of nephrotic syndrome with levamisole. J Pediatr 96: 490–493PubMedCrossRefGoogle Scholar
  4. 4.
    Mehta K, Ali U (1987) New approach to relapsing nephrotic syndrome. Pediatr Nephrol 1: C46CrossRefGoogle Scholar
  5. 5.
    Mongeau JG, Robitaille PO, Roy F (1988) Clinical efficacy of levamisole in the treatment of primary nephrosis in children. Pediatr Nephrol 2: 398–401PubMedCrossRefGoogle Scholar
  6. 6.
    Meyrier A, Simon P, Perret G, Condamin-Meyrier MC (1986) Remission of idiopathic nephrotic syndrome after treatment with cyclosporine A. Br Med J [Clin Res] 292: 789–793CrossRefGoogle Scholar
  7. 7.
    Hoyer PF, Krull F, Brodehl J (1986) Cyclosporine in frequently relapsing minimal change nephrotic syndrome. Lancet II: 385Google Scholar
  8. 8.
    Capodicasa G, De Santo NG, Nuzzi F, Giordano C (1986) Cyclosporine A in nephrotic syndrome of childhood. A 14 month experience. Int J Pediatr Nephrol 7: 69–72PubMedGoogle Scholar
  9. 9.
    Tejani A, Butt K, Trachtman H, Suthanthiran M, Rosenthal CJ, Khawar MR (1988) Cyclosporine A induced remission of relapsing nephrotic syndrome in children. Kidney Int 33: 729–734PubMedCrossRefGoogle Scholar
  10. 10.
    Brandis M, Burghard R, Leititis J, Zimmerhackl B, Hildebrandt F, Helmcher U (1988) Cyclosporine A for treatment of nephrotic syndrome. Transplant Proc 20 (Suppl 4): 275–279PubMedGoogle Scholar
  11. 11.
    Waldo FB, Kohaut EC (1987) Therapy of focal segmental glomerulosclerosis with cyclosporine A. Pediatr Nephrol 1: 180–182PubMedCrossRefGoogle Scholar
  12. 12.
    Brodehl J, Hoyer PF (1989) Cyclosporin in idiopathic nephrotic syndrome of children. Am J Nephrol 9 (Suppl 1): 61–64PubMedCrossRefGoogle Scholar
  13. 13.
    Niaudet P, the French Society of Pediatric Nephrology. (to be published) Steroid resistant idiopathic nephrotic syndrome and cyclosporine. NephronGoogle Scholar

Copyright information

© Springer Japan 1991

Authors and Affiliations

  • Patrick Niaudet
  • Michel Broyer
  • Renée Habib
    • 1
  1. 1.Service de Néphrologie Pédiatrique and INSERM U192Hôpital Necker Enfants MaladesParisFrance

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