1. Outcomes of renal replacement therapy in boys with prune belly syndrome: findings from the ESPN/ERA-EDTA Registry
- Author
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Marjolein Bonthuis, Heather Maxwell, Enrico Verrina, Kai Rönnholm, Karlijn J. van Stralen, Hassib Chehade, Sergey Baiko, Giovanni Montini, Søren Schwartz Sørensen, Stefanie Weber, Jérôme Harambat, Beyza Doğanay Erdoğan, Kitty J Jager, Fatoş Yalçınkaya, Franz Schaefer, Tim Ulinski, Jaap W. Groothoff, Bordeaux population health (BPH), Université de Bordeaux (UB)-Institut de Santé Publique, d'Épidémiologie et de Développement (ISPED)-Institut National de la Santé et de la Recherche Médicale (INSERM), APH - Quality of Care, APH - Aging & Later Life, Medical Informatics, ARD - Amsterdam Reproduction and Development, Paediatric Nephrology, APH - Methodology, AGEM - Amsterdam Gastroenterology Endocrinology Metabolism, APH - Global Health, ACS - Pulmonary hypertension & thrombosis, Clinicum, Children's Hospital, Lastentautien yksikkö, and HUS Children and Adolescents
- Subjects
Nephrology ,Male ,medicine.medical_specialty ,Adolescent ,medicine.medical_treatment ,030232 urology & nephrology ,030204 cardiovascular system & hematology ,Kidney ,LEHA ,Cohort Studies ,03 medical and health sciences ,0302 clinical medicine ,Prune belly syndrome ,Interquartile range ,3123 Gynaecology and paediatrics ,Internal medicine ,Journal Article ,medicine ,Humans ,Prune Belly Syndrome ,Renal replacement therapy ,Registries ,Child ,Survival rate ,Children ,Dialysis ,Kidney transplantation ,Transplantation ,business.industry ,medicine.disease ,GENE ,Kidney Transplantation ,3. Good health ,Surgery ,Child, Preschool ,Europe ,Kidney/pathology ,Kidney Failure, Chronic/etiology ,Kidney Failure, Chronic/mortality ,Kidney Failure, Chronic/therapy ,Kidney Transplantation/statistics & numerical data ,Prune Belly Syndrome/complications ,Prune Belly Syndrome/mortality ,Renal Replacement Therapy/methods ,Renal Replacement Therapy/statistics & numerical data ,Survival Rate ,Treatment Outcome ,Pediatrics, Perinatology and Child Health ,Kidney Failure, Chronic ,Original Article ,[SDV.SPEE]Life Sciences [q-bio]/Santé publique et épidémiologie ,business - Abstract
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 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.
- Published
- 2018
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