LOW-DOSE CYCLOSPORINE IN PEDIATRIC FSGS
759
and an 80% remission rate was reported. One
patient developed Hodgkin’s lymphoma after 10
months of treatment, but none developed hyper-
tension or worsening of renal function. Our remis-
sion rates are similar without the need for intrave-
nous methylprednisolone.
segmental glomerulosclerosis with idiopathic nephrotic syn-
drome: Three types of clinical response. J Pediatr 101:40-45,
1
982
. Tarshish P, Tobin JN, Bernstein J, Edelmann CM:
6
Cyclophosphamide does not benefit patients with focal seg-
mental glomerulosclerosis. A report of the International
Study of Kidney Disease in Children. Pediatr Nephrol 10:590-
593, 1996
2
3,25
Some earlier studies
also suggested that
7
. Tejani A, Phadke K, Nicastri A, Adamson O, Chen CK,
response to CsA is limited in steroid-resistant
patients (0% to 36%) compared with steroid-
dependent patients (80% to 100%). Other studies
that included only patients with steroid-resistant
FSGS have shown a 57% to 100% response rate
Trachtman H, Tejani C: Efficacy of cyclophosphamide in
steroid-sensitive nephrotic syndrome with different morpho-
logical lesions. Nephron 41:170-173, 1985
8. Mendoza SA, Reznik VM, Griswold WR, Krensky
AM, Yorgin PD, Tune BM: Treatment of steroid-resistant
focal segmental glomerulosclerosis with pulse methylpred-
nisolone and alkylating agents. Pediatr Nephrol 4:303-307,
1
6,19
to CsA therapy.
In our study population, the
response rate was 100% in the steroid-dependent
group and 69% in the steroid-resistant group.
Moreover, 50% of steroid-resistant patients
achieved complete remission of NS. The 5 ste-
roid-resistant patients who reached ESRD were
also CsA resistant, but even 1 of those patients
had an initial complete remission that was lost
when the dosage was tapered (Table 1, patient 11).
We conclude that CsA is an effective medica-
tion for the treatment of children with steroid-
resistant FSGS, and treatment success can be
achieved with a low dosage of oral CsA, 5
mg/kg/d, administered once daily to minimize
side effects of CsA therapy. Because most pa-
tients treated with CsAfor steroid-resistant FSGS
remain CsA dependent, the lowest effective CsA
dose should be used to avoid side effects of
long-term use. Single daily dose administration
may also improve patient and/or parent compli-
ance and lead to more successful treatment out-
comes with CsA.
1
990
9. Tune BM, Kirpekar R, Sibley RK, Reznik VM, Gris-
wold WR, Mendoza SA: Intravenous methylprednisolone
and oral alkylating agent therapy of prednisone resistant
pediatric focal segmental glomerulosclerosis: A long term
follow-up. Clin Nephrol 43:84-88, 1995
1
0. Tune BM, Mendoza SA: Treatment of the idiopathic
nephrotic syndrome: Regimens and outcomes in children
and adults. J Am Soc Nephrol 8:824-832, 1997
11. Sharma R, Sharma M, Ge X, McCarthy ET, Savin VJ:
Cyclosporine protects glomeruli from FSGS factor via an
increase in glomerular cAMP. Transplantation 62:1916-
1
920, 1996
2. Tune BM, Lieberman E, Mendoza SA: Steroid-
1
resistant nephrotic focal segmental glomerulosclerosis: A
treatable disease. Pediatr Nephrol 10:772-778, 1996
1
3. Gregory MJ, Smoyer WE, Sedman A, Kershaw DB,
Valentini RP, Johnson K, Bunchman TE: Long-term cyclo-
sporine therapy for pediatric nephrotic syndrome: A clinical
and histologic analysis. J Am Soc Nephrol 7:543-549, 1996
14. Zietse R, Derkx FH, Schalekamp MA, Weimar W:
Cyclosporine and the glomerular filtration barrier in mini-
mal change disease and membranous glomerulopathy. Con-
trib Nephrol 114:6-18, 1995
1
5. Borel JF, Feurer C, Gubler HU, Stahelin H: Biologi-
cal effects of cyclosporine A: A new anti-lymphocytic agent:
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