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Article Review/Hyperlink
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Gregory MJ, Smoyer WE, Sedman A, Kershaw DB, Valentini RP,
Johnson K, Bunchman TE
Long-term cyclosporine therapy for pediatric nephrotic
syndrome: a clinical histological analysis
J Am Soc Nephrol
(Apr) 7:543-549 1996

This is a study of children with idiopathic nephrotic syndrome
who were treated with a combination of cyclosporine and
alternate-day steroids for 6-53 months (mean 22). In 12 of 22
patients, a precyclosporine biopsy was performed as was a second
biopsy at 12-41 months, mean 21 months, later. They found that
in 2 patients, 17%, there was increase in interstitial fibrosis
and tubular atrophy suggesting cyclosporine nephrotoxicity.
Cyclosporine trough levels were maintained between 70 and 120
ng/ml. This low toxicity rate is the most important finding in
this study.
The response rate in patients with steroid resistant nephrotic
syndrome, 13 of 15, is more difficult to interpret. Most
pediatric nephrologists have several frustrating steroid
resistant patients with unremitting focal segmental
glomerulosclerosis (FSGS). The present study showed that 3 or 3
steroid resistant patients with FSGS responded to cyclosporine
and alternate day prednisone. The authors defined steroid
resistance as patients who have not entered remission after at
least 8 weeks of daily prednisone therapy. There is mounting
evidence that cyclosporine should not be instituted until at
least 6 months of daily prednisone has been administered. Many
"steroid resistant" patients become steroid sensitive if
prednisone is continued longer than 8 weeks. Therefore, it is
difficult to interpret the high remission rate the authors
observed in patients who were treated with cyclosporine and
labeled steroid resistant in this study. Many of these patients
might have benefitted from longer term steroid therapy.
Again, a major contribution of this study is the serial biopsy
material in 12 patients, which suggested a low incidence of
cyclosporine nephrotoxicity.
Don Moel, M.D., Loyola University at Chicago School of
Medicine
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