RECURRENT FOCAL SEGMENTAL GLOMERULOSCLEROSIS IN PEDIATRIC RENAL-TRANSPLANT RECIPIENTS - SUCCESSFUL TREATMENT WITH ORAL CYCLOPHOSPHAMIDE

Focal segmental glomerulosclerosis (FSGS) is the most common glomerulo pathy leading to end-stage renal disease in children and transplantati on is complicated by recurrent disease in a significant percentage of children. Treatment of recurrent FSGS has included high-dose steroids, high-dose cyclosporine (CSA), plasmapheresis, and ACE inhibitors with mixed results. We have had a consistent approach using oral cyclophos phamide (CTX) to treat recurrent FSGS since 1982. Three patients with ESRD secondary to nephrotic syndrome had recurrent disease. Biopsies i n all 3 were consistent with recurrent FSGS. Patients were begun on a 8-12 week course of 1-2 mg/kg/day of CTX and dosage was adjusted for W BC count. Azathioprine was with held during CTX. Patients' dosage at t he end of 12 weeks ranged from 0.89-1.75 mg/kg/day. All patients toler ated CTX well. After 8-12 weeks of treatment, 2 patients with nephroti c syndrome normalized their serum albumin and had negative to trace pr otein on urinary dipstick. One patient with proteinuria decreased his protein excretion from 770 to 340 mg/m2/day. At follow-up at 8, 38, an d 125 months post-transplant, these 3 patients have stable graft funct ion and negative to trace protein on urinalysis. The patient followed for 125 months has had 2 additional relapses at 51 and 82 months post- transplant that were treated successfully with pulse intravenous stero ids. Three pediatric patients with recurrent focal segmental glomerulo sclerosis post-renal transplant were treated with oral CTX and had sig nificant improvement in proteinuria and preservation of graft function . This suggests that oral CTX is a potentially effective and well-tole rated treatment for recurrent FSGS in children.