Outcomes of renal replacement therapy in boys with prune belly syndrome: findings from the ESPN/ERA-EDTA Registry.

Details

Ressource 1Download: 28779237_BIB_E95928B10BFE.pdf (814.57 [Ko])
State: Public
Version: Final published version
License: CC BY 4.0
Serval ID
serval:BIB_E95928B10BFE
Type
Article: article from journal or magazin.
Collection
Publications
Institution
Title
Outcomes of renal replacement therapy in boys with prune belly syndrome: findings from the ESPN/ERA-EDTA Registry.
Journal
Pediatric nephrology
Author(s)
Yalcinkaya F., Bonthuis M., Erdogan B.D., van Stralen K.J., Baiko S., Chehade H., Maxwell H., Montini G., Rönnholm K., Sørensen S.S., Ulinski T., Verrina E., Weber S., Harambat J., Schaefer F., Jager K.J., Groothoff J.W.
ISSN
1432-198X (Electronic)
ISSN-L
0931-041X
Publication state
Published
Issued date
01/2018
Peer-reviewed
Oui
Volume
33
Number
1
Pages
117-124
Language
english
Notes
Publication types: Journal Article
Publication Status: ppublish
Abstract
As outcome data for prune belly syndrome (PBS) complicated by end-stage renal disease are scarce, we analyzed characteristics and outcomes of children with PBS using the European Society for Pediatric Nephrology/European Renal Association-European Dialysis and Transplant Association (ESPN/ERA-EDTA) Registry data.
Data were available for 88 male PBS patients aged <20 years who started renal replacement therapy (RRT) between 1990 and 2013 in 35 European countries. Patient characteristics, survival, and transplantation outcomes were compared with those of male patients requiring RRT due to congenital obstructive uropathy (COU) and renal hypoplasia or dysplasia (RHD).
Median age at onset of RRT in PBS was lower [7.0; interquartile range (IQR) 0.9-12.2 years] than in COU (9.6; IQR: 3.0-14.1 years) and RHD (9.4; IQR: 2.7-14.2 years). Unadjusted 10-year patient survival was 85% for PBS, 94% for COU, and 91% for RHD. After adjustment for country, period, and age, PBS mortality was similar to that of RHD but higher compared with COU [hazard ratio (HR) 1.96, 95% confidence interval (CI) 1.03-3.74]. Seventy-four PBS patients (84%) received a first kidney transplant after a median time on dialysis of 8.4 (IQR 0.0-21.1) months. Outcomes with respect to time on dialysis before transplantation, chance of receiving a first transplant within 2 years after commencing RRT, and death-censored, adjusted risk of graft loss were similar for all groups.
This study in the largest cohort of male patients with PBS receiving RRT to date demonstrates that outcomes are comparable with other congenital anomalies of the kidney and urinary tract, except for a slightly higher mortality risk compared with patients with COU.
Keywords
Adolescent, Child, Child, Preschool, Cohort Studies, Europe, Humans, Kidney/pathology, Kidney Failure, Chronic/etiology, Kidney Failure, Chronic/mortality, Kidney Failure, Chronic/therapy, Kidney Transplantation/statistics & numerical data, Male, Prune Belly Syndrome/complications, Prune Belly Syndrome/mortality, Registries, Renal Replacement Therapy/methods, Renal Replacement Therapy/statistics & numerical data, Survival Rate, Treatment Outcome, Children, Dialysis, Prune belly syndrome, Renal replacement therapy, Transplantation
Pubmed
Web of science
Open Access
Yes
Create date
05/09/2017 16:29
Last modification date
20/08/2019 16:11
Usage data