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

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  1. Demographics of paediatric renal replacement therapy in Europe: a report of the ESPN/ERA-EDTA registry

    Research output: Contribution to journalJournal articleResearchpeer-review

  2. Branchio-oto-renal syndrome caused by partial EYA1 deletion due to LINE-1 insertion

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  3. Demographics of paediatric renal replacement therapy in Europe: 2007 annual report of the ESPN/ERA-EDTA registry

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  4. Increased kidney growth in formula-fed versus breast-fed healthy infants

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  5. Kidney growth in 717 healthy children aged 0-18 months: a longitudinal cohort study

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  • Fatos Yalcinkaya
  • Marjolein Bonthuis
  • Beyza Doganay Erdogan
  • Karlijn J van Stralen
  • Sergey Baiko
  • Hassib Chehade
  • Heather Maxwell
  • Giovanni Montini
  • Kai Rönnholm
  • Søren Schwartz Sørensen
  • Tim Ulinski
  • Enrico Verrina
  • Stefanie Weber
  • Jérôme Harambat
  • Franz Schaefer
  • Kitty J Jager
  • Jaap W Groothoff
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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.

Original languageEnglish
JournalPediatric nephrology (Berlin, Germany)
Volume33
Issue number1
Pages (from-to)117-124
Number of pages8
ISSN0931-041X
DOIs
Publication statusPublished - 2018

    Research areas

  • Journal Article

ID: 52424793