Renal transplantation in children with nephrotic syndrome in the first year of life

Objective: The aim of this work was to analyze the evolution of the 1st renal transplantation in children with nephrotic syndrome in the 1st year of life (NSFL).

Methods: In this retrospective study of 15 patients (8 women and 7 men) with NSFL receiving transplants from 1989 to 2013, 9 had NS of Finnish type, 4 diffuse mesangial sclerosis, 1 minimal changes, and 1 collapsing glomerulopathy. We analyzed the clinical and analytic situation at 4 time points: before dialysis, before transplantation, 3 months after transplantation, and long-term evolution.

Results: Mean follow-up was 72.8 months (range, 1 month to 16.9 years); mean age at diagnosis was 2.21 months (range, 0-8.2 months); mean age at onset of replacement therapy was 22.9 ± 16.4 months (range, 3.8-55.4 months); and mean time on dialysis was 14.9 months (range, 2-44 months). Age at transplantation was 3.1 years (range, 1.8 to 7.7 years), with 6 living-donor transplantations (LDTs) and 9 cadaveric (CDTs). Ten patients required nephrectomy before transplantation (9 bilateral) to control proteinuria after 3.1 ± 3.8 months on dialysis, 1 during transplantation, and 3 after transplantation (2 persistent proteinuria, 1 hypertension). Mean time on dialysis for LDTs was 5.4 ± 2.7 months versus 13.2 ± 6.9 months for CDTs (P < .005). Mean age of cadaveric donors was 6.2 ± 2.4 years and that of living donors 35.5 ± 7.9 years. As complications, there was 1 bleeding from venous anastomosis and 1 urinary leakage after surgery. After 6 ± 5.2 years of evolution, actuarial survival at both 1 and 7 years was 92.9%. One graft was lost owing to acute rejection 1 month after transplantation and 2 others owing to chronic rejection >9 years after transplantation. None had disease recurrence.

Conclusions: Short-term complications did not differ from the rest of population if transplantation occurred with standard albumin levels, for which most required pre-transplantation nephrectomy because dialysis failed to reduce proteinuria.