Recurrence of focal segmental glomerulosclerosis (FSGS) after renal transplantation can limit graft survival. Despite new immunosuppressive agents, the incidence of recurrence remains relatively high. To identify risk factors for recurrence and efficacy of treatment, we reviewed the outcome of 23 grafts in 16 children with FSGS who had undergone transplantation between 1985 and 2007 at La Paz Children's Hospital. Recurrence was 56.3% after the first transplantation. We did not find significant differences in age at diagnosis, age at transplantation, age at end-stage renal disease (ESRD), progression to ESRD, bilateral nephrectomy of native kidneys prior to transplantation, use of induction therapy or of different immunosuppressive regimens between patients with and without recurrence. Plasmapheresis (PP) was carried out in seven of nine patients who had suffered recurrence, achieving remission in six of them. One patient received high doses of cyclosporin (CsA) and plasmapheresis, attaining remission. Graft survival was lower (P = 0.043) in patients with FSGS than in those with other ESRD etiologies (first year 75% vs 91%; fifth year 44% vs 78%). Recurrence of FSGS limited graft survival (first year 66% vs 85%; third year 20% vs 68%) (P = 0.07). In our experience, PP can be effective in treating FSGS recurrence, although its effect on long-term graft survival seems more limited.

Fourteen patients with posterior urethral valves and end-stage renal failure were urodynamically evaluated in order to identify and correct any bladder dysfunction before renal transplant. Of the 14 patients, during filling, six had normal bladder function, two had an over-distended bladder (one with instability), one had instability, four had poor compliance, and one had a very reduced bladder capacity. During the voiding phase, one had a myogenic failure and another had detrusor-sphincteric dyssynergia. Four patients underwent bladder augmentation. Three were managed with anticholinergic therapy and two required clean intermittent catheterization owing to post-voiding residual urine. A renal transplant was performed in all of these patients at a mean age of 8.2 years. We compared outcome in these 14 patients with bladder dysfunction treated before transplantation with outcome in a matched control group of 14 transplant patients. Graft function and survival were similar in both groups. We believe that urodynamic studies must be included in the pretransplant evaluation of patients with posterior urethral valves in order to diagnose any bladder dysfunction and commence appropriate treatment to avoid any negative influence on graft function.

We present a retrospective study of 30 children of mean age 3.02 +/- 1.81 years with steroid-resistant nephrotic syndrome (SRNS) treated with intravenous injection of methylprednisolone plus orally administered prednisone; 24 children also received cyclophosphamide (CP). Sixteen were resistant to steroids from the beginning, and 14 after a mean of 11.26 +/- 16.61 months. The initial histological diagnosis was: 18 minimal change disease (MCD), 11 focal segmental glomerulosclerosis (FSGS) and one diffuse mesangial proliferative glomerulonephritis (DMPG). Total remission was achieved in 22 patients (73.3%), partial response in three (10%) and no response in five (16.6%), two of whom were brothers carrying an NPHS2 gene double mutation. There was no difference in response between the MCD and FSGS patients; the only patient with DMPG did not respond. Only initial resistance was a sign of bad prognosis. At follow-up (6.4 +/- 3.6 years from last pulse), 21/22 were still in remission, 14/21 were without treatment. Six patients required cyclosporine or mycophenolate mofetil because of steroid dependence. Two non-responders developed end-stage renal failure (ESRF); the remaining patients maintained normal glomerular filtration. The treatment was well tolerated. In conclusion, most of the patients treated with sequential therapy consisting of methylprednisolone (MP) (100%) and CP (80%) showed remission and preserved renal function, but 20% developed steroid dependence.

Both patient and allograft survival proved to be excellent in paediatric patients (98.4% and 90.3%) and adults (96.8% and 95.0%). The rates of biopsy-proven acute rejections (BPAR) and treated acute rejection episodes (ARE) were comparable between paediatric (12.9% and 17.7%) and adult patients (15.5% and 20.7%). Transplant function at 12 months post-transplant was similar in paediatric (67.8 ± 45.6 mL/min/1.73 m2;) and adult recipients (64.7 ± 23.3 mL/min/1.73 m2;). Children < 6 years (n = 10) exhibited a numerically higher frequency of leucocytopaenia (20%), diarrhoea (40%) and weight loss (10%) than older children (6-18 years; 5.8%, 28.8% and 1.9%) and adults (16.1%, 24.7% and 1.5%). On the whole, the percentage of patients who experienced adverse events causing interruption of MMF therapy were numerically lower in children (4.8%) than in adults (12.5%). Conclusions. The overall efficacy and tolerability of MMF appear to be comparable between paediatric and adult patients. Further studies are needed to validate these results.

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