Early or Late Transplantation in Congenital Nephrotic Syndrome: Which is Effective for Optimal Growth?


TRANSPLANTATION PROCEEDINGS, vol.51, no.7, pp.2283-2288, 2019 (SCI-Expanded) identifier identifier identifier


Congenital nephrotic syndrome (CNS) is a genetic disease that is present in the antenatal period or during the first 3 months of life. In this study, we aimed to compare growth parameters of patients with CNS who received kidney transplants and either (1) had a normal glomerular filtration rate (GFR) at the time of transplant or (2) chronic kidney disease (CKD) at the time of transplant. Patients with a diagnosis of CNS who had a minimum follow-up period of 6 months were evaluated retrospectively. Children at stages 4 or 5 CKD or patients receiving dialysis during the pretransplant period were defined as group 1; patients with normal GFR at the time of transplantation were classified as group 2. Short stature and low weight were defined as less than -2 standard deviation scores (SDS) for height and weight according to their age. A total of 17 patients were included in the study. Thirteen of 17 patients had NPHS1 gene mutations. Group 1 and group 2 consisted of 8 and 9 patients, respectively. Mean height SDS and mean weight SDS in group 2 were higher than group 1 in the pre-transplant period (-4.34 +/- 1.74 vs -2.84 +/- 1.56; P =.011 and -3.54 +/- 0.93 vs -1.83 +/- 1.13; P =.008). In the post-transplant period, the significant difference in height SDS continued in favor of group 2 (-3.16 +/- 1.11 vs 1.16 +/- 0.87; P =.002). The short stature rate was 83% in group 1 and 72% in group 2 in the pretransplant period (P = .62), and 83% in group 1 and 27% in group 2 in the post-transplant period (P = .02). Early renal transplantation seems to be effective for optimal height gain in children with CNS.