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

Transplant Proc. 2019 Sep;51(7):2283-2288. doi: 10.1016/j.transproceed.2019.01.191. Epub 2019 Aug 7.

Abstract

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 pretransplant 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.

MeSH terms

  • Adolescent
  • Body Height
  • Body Weight
  • Child
  • Child, Preschool
  • Female
  • Glomerular Filtration Rate
  • Growth Disorders / etiology*
  • Humans
  • Kidney Transplantation / methods
  • Kidney Transplantation / statistics & numerical data*
  • Male
  • Nephrotic Syndrome / physiopathology
  • Nephrotic Syndrome / surgery*
  • Postoperative Complications / etiology*
  • Postoperative Period
  • Renal Dialysis / statistics & numerical data
  • Renal Insufficiency, Chronic / congenital
  • Renal Insufficiency, Chronic / physiopathology
  • Renal Insufficiency, Chronic / surgery*
  • Retrospective Studies
  • Time Factors*

Supplementary concepts

  • Nephrosis, congenital