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Am J Kidney Dis. 2004 Jul;44(1):50-6.

High-dose oral cyclosporin therapy for recurrent focal segmental glomerulosclerosis in children.

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1
Division of Pediatric Nephrology, University of Texas Health Science Center San Antonio, San Antonio, TX, USA.

Abstract

BACKGROUND:

Focal segmental glomerular sclerosis (FSGS) has a high propensity for recurrence after renal transplantation, with a 50% risk for graft failure from recurrent disease.

METHODS:

We report on the efficacy of high-dose oral cyclosporin A (CsA) in the treatment of recurrent FSGS in children. Between August 1991 and January 2003, a total of 24 patients with FSGS underwent transplantation at 1 institution. Sixteen patients (67%) had recurrent disease. In these 16 patients, CsA dose was increased gradually until remission was achieved or there was evidence of renal toxicity. Seven patients also underwent plasma exchange.

RESULTS:

Thirteen patients (81%) achieved remission. Remission was complete in 11 patients and partial in 2 patients. The CsA dose necessary for inducing remission ranged from 6 to 25 mg/kg/d. Four of these patients also underwent plasma exchange. After remission, CsA dose was reduced gradually toward the standard posttransplantation regimen. Eleven of 13 responders have a functioning graft after a follow-up ranging from 10 months to 12 years. One graft was lost because of recurrent FSGS, and another graft because of recurrence and cellular rejection; noncompliance was a factor in both losses. The 3 patients with disease that did not respond to high-dose CsA therapy lost their grafts because of recurrent FSGS. A common factor in these 3 patients was the inability to increase the CsA dose because of early evidence of nephrotoxicity, evidenced by an increase in serum creatinine level.

CONCLUSION:

High-dose oral CsA therapy is effective in producing long-lasting remission of recurrent nephrotic syndrome in children with FSGS.

PMID:
15211437
[Indexed for MEDLINE]
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