Ciclosporin Treatment in Children with Steroid-dependent Nephrotic Syndrome

Overview

Journal Pediatr Nephrol

Specialties Nephrology
Pediatrics

Date 1990 Sep 1

PMID 2242308

Citations 12

Authors

Y Kitano

N Yoshikawa

R Tanaka

H Nakamura

M Ninomiya

H Ito

Affiliations

Soon will be listed here.

Abstract

We report our experience with ciclosporin (CS) treatment in 18 children with steroid-dependent nephrotic syndrome. CS was started at 3-5 mg/kg per day after the patients had attained remission with steroid therapy, and was adjusted to maintain a trough blood level of between 250 and 600 ng/ml, being administered for 6 months. Although 1 patient dropped out of the study because of renal dysfunction, the remaining 17 children completed the full trial. No relapse occurred during the 6-month period of CS treatment, and it was possible to discontinue steroid therapy in all patients. However, after discontinuation of CS treatment, nephrotic syndrome relapsed in 16 patients and 14 again had frequent relapses and became steroid-dependent, as before CS treatment. The effect of CS in maintaining remission from steroid-dependent nephrotic syndrome was thus dependent on continuation of CS treatment. Although several side-effects occurred during CS treatment, they were not so serious as to necessitate discontinuation of treatment, except in 1 patient, and all of these side effects were reversible. CS is therefore a new agent for the management of children with steroid-dependent nephrotic syndrome in place of corticosteroid and alkylating agents, although long-term maintenance therapy may be necessary for maintaining longer remission.

Citing Articles

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References

Makker S, HEYMANN W . The idiopathic nephrotic syndrome of childhood. A clinical reevaluation of 148 cases. Am J Dis Child. 1974; 127(6):830-7. DOI: 10.1001/archpedi.1974.02110250056008. View

Garin E, Pryor N, Fennell 3rd R, RICHARD G . Pattern of response to prednisone in idiopathic, minimal lesion nephrotic syndrome as a criterion in selecting patients for cyclophosphamide therapy. J Pediatr. 1978; 92(2):304-8. DOI: 10.1016/s0022-3476(78)80032-8. View

. Cyclophosphamide treatment of steroid dependent nephrotic syndrome: comparison of eight week with 12 week course. Report of Arbeitsgemeinschaft für Pädiatrische Nephrologie. Arch Dis Child. 1987; 62(11):1102-6. PMC: 1778533. DOI: 10.1136/adc.62.11.1102. View

SHALHOUB R . Pathogenesis of lipoid nephrosis: a disorder of T-cell function. Lancet. 1974; 2(7880):556-60. DOI: 10.1016/s0140-6736(74)91880-7. View

. Early identification of frequent relapsers among children with minimal change nephrotic syndrome. A report of the International Study of Kidney Disease in Children. J Pediatr. 1982; 101(4):514-8. DOI: 10.1016/s0022-3476(82)80692-6. View

Niaudet P, Habib R, Tete M, HINGLAIS N, Broyer M . Cyclosporin in the treatment of idiopathic nephrotic syndrome in children. Pediatr Nephrol. 1987; 1(4):566-73. DOI: 10.1007/BF00853590. View

Brandis M, Burghard R, Leititis J, Zimmerhackl B, Hildebrandt F, Helmchen U . Cyclosporine A for treatment of nephrotic syndromes. Transplant Proc. 1988; 20(3 Suppl 4):275-9. View

Mallick N . The pathogenesis of minimal change nephropathy. Clin Nephrol. 1977; 7(3):87-95. View

Siegel N, Goldberg B, Krassner L, HAYSLETT J . Long-term follow-up of children with steroid-responsive nephrotic syndrome. J Pediatr. 1972; 81(2):251-8. DOI: 10.1016/s0022-3476(72)80291-9. View

10.

BRODEHL J, Hoyer P, Oemar B, Helmchen U, Wonigeit K . Cyclosporine treatment of nephrotic syndrome in children. Transplant Proc. 1988; 20(3 Suppl 4):269-74. View

11.

Ponticelli C, Rivolta E . Cyclosporine in nephrotic syndrome. Transplant Proc. 1988; 20(3 Suppl 4):253-8. View

12.

Arneil G, Lam C . Long-term assessment of steroid therapy in childhood nephrosis. Lancet. 1966; 2(7468):819-21. DOI: 10.1016/s0140-6736(66)92253-7. View

13.

. Primary nephrotic syndrome in children: clinical significance of histopathologic variants of minimal change and of diffuse mesangial hypercellularity. A Report of the International Study of Kidney Disease in Children. Kidney Int. 1981; 20(6):765-71. DOI: 10.1038/ki.1981.209. View

14.

Shevach E . The effects of cyclosporin A on the immune system. Annu Rev Immunol. 1985; 3:397-423. DOI: 10.1146/annurev.iy.03.040185.002145. View

15.

. Effect of cytotoxic drugs in frequently relapsing nephrotic syndrome with and without steroid dependence. N Engl J Med. 1982; 306(8):451-4. DOI: 10.1056/NEJM198202253060803. View

16.

Capodicasa G, De Santo N, Nuzzi F, Giordano C . Cyclosporin A in nephrotic syndrome of childhood--a 14 month experience. Int J Pediatr Nephrol. 1986; 7(2):69-72. View

17.

Tejani A, Butt K, Trachtman H, Suthanthiran M, Rosenthal C, Khawar M . Cyclosporine-induced remission of relapsing nephrotic syndrome in children. J Pediatr. 1987; 111(6 Pt 2):1056-62. DOI: 10.1016/s0022-3476(87)80056-2. View

18.

Schwartz M, Schwartz G, Cornfeld D . A 16-year follow-up study of 163 children with nephrotic syndrome. Pediatrics. 1974; 54(5):547-52. View