ESPN 51th Annual Meeting

ESPN 2018


 
CONGENITAL NEPHROTIC SYNDROME: TRANSPLANTATION BEFORE OR AFTER CHRONIC RENAL FAILURE DEVELOPMENT?
GULSAH KAYA AKSOY 1 MUSTAFA KOYUN 1 ELIF COMAK 1 SEMA AKMAN 1

1- AKDENIZ UNIVERSITY, MEDICAL FACULTY, DEPARTMENT OF PEDIATRICS, DIVISION OF PEDIATRIC NEPHROLOGY, ANTALYA, TURKEY
 
Introduction:

Congenital nephrotic syndrome (CNS) is a genetic disease that is present in the antenatal period or during the first 3months of life. In this study, we aimed to compare growth parameters of patients with CNS who were transplanted with normal glomerular filtration rate (GFR) and that after ESRD developed.

Material and methods:

Patients with a diagnosis of CNS who underwent renal transplantation between 2005 and 2017 and who had a minimum follow-up period of 6 months were evaluated retrospectively. Children at Stage 4-5 CKD or patients receiving dialysis at pre-transplant period were defined as group 1; patients with normal glomerular filtration rate (GFR) at the time of transplantation were classified as group 2. Data on weight and height were recorded; weight and height standard deviation scores (SDS) were calculated. Short stature and low weight was defined as below -2 SDS for height and weight according to their age, respectively.

Results:

A total of 17 patients, 10 boys and 7 girls, were included in the study. 13 of 17 patients had NPHS1 gene mutations. All patients had normal graft function at the time of study. Group 1 and group 2 consisted of 8 and 9 patients, respectively. The median period between diagnosis and transplantation, and median follow-up period after transplantation was similar between group1 and 2 (3.65 vs 1.98 years, p=0.236 and 1.08 vs 3.25 years, p=0.09, respectively). Mean height SDS of group 2 was higher than group 1 at both pre-transplant and post-transplant period (-2.31±1.04 vs -4.57±1.60, p=0.011 and -1.16±0.87 vs -3.16±1.11, p=0.002). Short stature rate was higher in group 1 than group 2 at post-transplant period (87.5% vs 11.1%, p=0.001), whereas it was similar at pre-transplant period (87.5% vs 33.3%, p=0.31). Patients with low weight rate was higher in group 1 than group 2 both at pre-transplant (100.0% vs 44.4%, p=0.012), and at post-transplant period (50.0% vs 0%, p=0.015, respectively).

Conclusions:

Early renal transplantation seems to be effective for optimal height and weight gain in children with congenital nephrotic syndrome.