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

• Published in: Pediatric nephrology (Berlin, West) Vol. 33; no. 1; pp. 117 - 124
• Main Authors: Yalcinkaya, Fatos, Bonthuis, Marjolein, Erdogan, Beyza Doganay, van Stralen, Karlijn J., Baiko, Sergey, Chehade, Hassib, Maxwell, Heather, Montini, Giovanni, Rönnholm, Kai, Sørensen, Søren Schwartz, Ulinski, Tim, Verrina, Enrico, Weber, Stefanie, Harambat, Jérôme, Schaefer, Franz, Jager, Kitty J., Groothoff, Jaap W.
• Format: Journal Article
• Language: English
• Published: Berlin/Heidelberg Springer Berlin Heidelberg 01.01.2018; Springer; Springer Nature B.V; Springer Verlag
• Subjects: Adolescent; Child; Child, Preschool; Children; Cohort Studies; Congenital defects; Dialysis; Dysplasia; Edetic acid; End-stage renal disease; Europe; Hemodialysis; Humans; Hypoplasia; Kidney - pathology; Kidney diseases; Kidney Failure, Chronic - etiology; Kidney Failure, Chronic - mortality; Kidney Failure, Chronic - therapy; Kidney transplantation; Kidney Transplantation - statistics & numerical data; Life Sciences; Male; Medicine; Medicine & Public Health; Mortality; Nephrology; Original; Original Article; Patient outcomes; Patients; Pediatrics; Prune belly syndrome; Prune Belly Syndrome - complications; Prune Belly Syndrome - mortality; Registries; Renal replacement therapy; Renal Replacement Therapy - methods; Renal Replacement Therapy - statistics & numerical data; Santé publique et épidémiologie; Survival; Survival Rate; Transplantation; Transplants & implants; Treatment Outcome; Urinary tract; Urology; Transplantation; Dialysis; Children; Renal replacement therapy; Prune belly syndrome; LEHA
• ISSN: 0931-041X; 1432-198X
• DOI: 10.1007/s00467-017-3770-9

Background 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. Methods 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). Results 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. Conclusions 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.