[Long-term monitoring of children with focal segmental glomerulosclerosis and a transplanted kidney]. / Dugotrajno pracenje djece s fokalnom segmentalnom glomerulosklerozom i presadenim bubregom.

ABSTRACT

INTRODUCTION: Focal segmental glomerulosclerosis (FSGS) is an important cause of nephrotic syndrome and end-stage renal disease. Children with FSGS are at a risk of recurrence of disease following renal transplantation. The rate of recurrence has been estimated to range from 15% to 50%, having a potentially detrimental course towards the loss of renal function. The factors associated with an increased probability of recurrence are not well known. PATIENTS The authors followed 8 pediatric patients (3 girls and 5 boys) with FSGS who had undergone renal transplantation (16% of all transplanted pediatric patients) over a long period (1982-2001). The mean age of the children at the time of disease onset was 4 years and 8 months (range 2 months-12 years). Children were monitored from 10 months to 4 years before the first dialysis. On average, the first dialysis was performed at 7 years of age (range 12 months-16 years). Time elapsed between the first dialysis and transplantation ranged from 1 to 3 years (mean 1.5 year), and mean age at transplantation was 8 years and 6 months (range 4-18 years). The grafts were from 2 living-related and 6 cadaveric donors. Five recipients were immunosuppressed with cyclosporin A (CsA) -steroids-azathioprine (Aza), 2 with CsA-steroids-mycophenolate mofetil (MMF), and 1 with CsA-steroids regimen. Follow-up period after transplantation ranged from 2 to 15 years (mean time 7 years and 8 months). RESULTS: Creatinine values, proteinuria range and blood pressure were monitored every 3 months after transplantation, and were as follows creatinine 41-386 mumol/l (mean value 153 mumol/l), proteinuria 0.01-3,14 g/l (mean 0.27 g/l, median 0.03 g/l), systolic blood pressure 90-140 mm Hg (mean 110 mm Hg), diastolic blood pressure 60-90 mm Hg (mean 80 mm Hg). Two patients developed hypertension grade I and III after renal transplantation. There were 5 (0.6 per patient) acute rejection episodes. Two grafts (25%) were rejected 5 and 9 years after transplantation, but recurrence of FSGS was not confirmed by renal biopsy. CONCLUSION: Due to the small sample size no firm conclusions about recurrence of FSGS could be made. However, the fact that none of 8 children developed recurrence of FSGS after renal transplantation speaks against the relatively high recurrence rates in our pediatric population.