Twenty-two renal transplants were performed in 21 children less than two years of age at Children's Hospital. Fourteen were from living related donors and eight were from cadaveric donors. The five year patient and graft survivals of these recipients were compared to all other pediatric recipients between two and 18 years of age who received renal transplants over the same time period. Five year graft survival for recipients less than two years of age was 86% following living-related donor transplantation and 38% following cadaver donor transplantation. Older pediatric recipients aged between two and 18 years had a five year graft survival of 73% following living-related donor renal transplantation, which was similar to that for recipients less than two years of age. Although older cadaveric recipients had a comparable five year graft survival to younger recipients, at 42%, the patterns of graft loss were different. Graft failures in young recipients occurred within the first seven months post-transplant, whereas the older recipient's grafts failed more gradually. Actuarial five-year patient survival in recipients less than two years of age was 86% following living-related donor renal transplantation and 70% following cadaver-donor renal transplantation. Recipients less than two years of age had a poorer patient survival than older recipients following both living-related donor renal transplantation (P = 0.06) and cadaver-donor renal transplantation (P less than 0.05). These findings suggest that the graft survival of living-related donor renal transplantation in recipients less than two years of age is better than that of cadaver-donor renal transplantation.(ABSTRACT TRUNCATED AT 250 WORDS)

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Congenital nephrotic syndrome (CNS) is a rare and uniformly fatal disease if it is not treated. Although renal transplantation has been a successful treatment, there remains a high mortality rate during the first year of life before transplantation. From 1979 to 1987, four patients with CNS, all of whom died before they could undergo renal transplantation were treated. On the basis of this clinical experience, early elective bilateral nephrectomy and dialysis for infants with CNS were initiated to improve overall outcome. From 1987 to 1989, this protocol has been used on four consecutive patients with CNS. In these patients, bilateral nephrectomy was performed only after patients suffered a serious CNS-related complication that occurred between 4 and 6 months of age. Despite nephrectomy and the need for chronic dialysis, all patients grew at normal or accelerated rates. By 16 months of age, all patients underwent successful renal transplantation.