. 2018 Jan;33(1):117-124.

doi: 10.1007/s00467-017-3770-9. Epub 2017 Aug 4.

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

Fatos Yalcinkaya¹, Marjolein Bonthuis², Beyza Doganay Erdogan³, Karlijn J van Stralen⁴, Sergey Baiko⁵, Hassib Chehade⁶, Heather Maxwell⁷, Giovanni Montini⁸, Kai Rönnholm⁹, Søren Schwartz Sørensen¹⁰, Tim Ulinski^{11

12}, Enrico Verrina¹³, Stefanie Weber¹⁴, Jérôme Harambat¹⁵, Franz Schaefer¹⁶, Kitty J Jager¹⁷, Jaap W Groothoff¹⁸

Affiliations

¹ Department of Pediatric Nephrology, Ankara University Faculty of Medicine, Ankara, Turkey.
² ESPN/ERA-EDTA Registry, Department of Medical Informatics, Academic Medical Center, University of Amsterdam, Amsterdam Public Health research institute, Amsterdam, The Netherlands. m.bonthuis@amc.uva.nl.
³ Department of Biostatistics, Ankara University School of Medicine, Ankara, Turkey.
⁴ Spaarne Gasthuis Academie, Spaarne Gasthuis, Hoofddorp, The Netherlands.
⁵ Department of Pediatrics, Belarusian State Medical University, Minsk, Belarus.
⁶ Department of Pediatrics, Division of Pediatric Nephrology, University Hospital, Lausanne, Switzerland.
⁷ Department of Paediatric Nephrology, Royal Hospital for Sick Children, Glasgow, UK.
⁸ Pediatric Nephrology and Dialysis Unit, Department of Clinical Sciences and Community Health, University of Milan Fondazione IRCCS Cà Granda, Ospedale Maggiore Policlinico Via della Commenda, 9, 20122, Milan, Italy.
⁹ Children's Hospital University of Helsinki, Helsinki, Finland.
¹⁰ Department of Nephrology, Rigshospitalet, Copenhagen University Hospital, Copenhagen, Denmark.
¹¹ Department of Paediatric Nephrology, Armand-Trousseau Hospital, APHP, Paris, France.
¹² University Pierre and Marie Curie, Paris, France.
¹³ Dialyisis Unit, Paediatric Nephrology and Dialysis Department, IRCCS Giannina Gaslini, Genoa, Italy.
¹⁴ University Children's Hospital Marburg, Philipps-University, Marburg, Germany.
¹⁵ Pediatric Nephrology Unit, Bordeaux University Hospital, Bordeaux, France.
¹⁶ Department of Pediatric Nephrology, University Children's Hospital Heidelberg, Heidelberg, Germany.
¹⁷ ESPN/ERA-EDTA Registry, Department of Medical Informatics, Academic Medical Center, University of Amsterdam, Amsterdam Public Health research institute, Amsterdam, The Netherlands.
¹⁸ Department of Pediatric Nephrology, Emma Children's Hospital, Academic Medical Center, Amsterdam, The Netherlands.

PMID: 28779237
PMCID: PMC5700229
DOI: 10.1007/s00467-017-3770-9

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

Fatos Yalcinkaya et al. Pediatr Nephrol. 2018 Jan.

. 2018 Jan;33(1):117-124.

doi: 10.1007/s00467-017-3770-9. Epub 2017 Aug 4.

Authors

Affiliations

¹ Department of Pediatric Nephrology, Ankara University Faculty of Medicine, Ankara, Turkey.
² ESPN/ERA-EDTA Registry, Department of Medical Informatics, Academic Medical Center, University of Amsterdam, Amsterdam Public Health research institute, Amsterdam, The Netherlands. m.bonthuis@amc.uva.nl.
³ Department of Biostatistics, Ankara University School of Medicine, Ankara, Turkey.
⁴ Spaarne Gasthuis Academie, Spaarne Gasthuis, Hoofddorp, The Netherlands.
⁵ Department of Pediatrics, Belarusian State Medical University, Minsk, Belarus.
⁶ Department of Pediatrics, Division of Pediatric Nephrology, University Hospital, Lausanne, Switzerland.
⁷ Department of Paediatric Nephrology, Royal Hospital for Sick Children, Glasgow, UK.
⁸ Pediatric Nephrology and Dialysis Unit, Department of Clinical Sciences and Community Health, University of Milan Fondazione IRCCS Cà Granda, Ospedale Maggiore Policlinico Via della Commenda, 9, 20122, Milan, Italy.
⁹ Children's Hospital University of Helsinki, Helsinki, Finland.
¹⁰ Department of Nephrology, Rigshospitalet, Copenhagen University Hospital, Copenhagen, Denmark.
¹¹ Department of Paediatric Nephrology, Armand-Trousseau Hospital, APHP, Paris, France.
¹² University Pierre and Marie Curie, Paris, France.
¹³ Dialyisis Unit, Paediatric Nephrology and Dialysis Department, IRCCS Giannina Gaslini, Genoa, Italy.
¹⁴ University Children's Hospital Marburg, Philipps-University, Marburg, Germany.
¹⁵ Pediatric Nephrology Unit, Bordeaux University Hospital, Bordeaux, France.
¹⁶ Department of Pediatric Nephrology, University Children's Hospital Heidelberg, Heidelberg, Germany.
¹⁷ ESPN/ERA-EDTA Registry, Department of Medical Informatics, Academic Medical Center, University of Amsterdam, Amsterdam Public Health research institute, Amsterdam, The Netherlands.
¹⁸ Department of Pediatric Nephrology, Emma Children's Hospital, Academic Medical Center, Amsterdam, The Netherlands.

PMID: 28779237
PMCID: PMC5700229
DOI: 10.1007/s00467-017-3770-9

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 <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.

Keywords: Children; Dialysis; Prune belly syndrome; Renal replacement therapy; Transplantation.

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Conflict of interest statement

We have no conflicts of interest to declare.

Figures

**Fig. 1**
Age distributions of patients at the onset of renal replacement therapy (RRT)

**Fig. 2**
Age at renal replacement therapy (RRT) onset over the two time periods. Only countries with complete follow-up are included in the analyses. Dotted lines indicate median age [interquartile range (IQR)] at start of RRT

**Fig. 3**
Unadjusted survival of patients with prune belly syndrome (PBS) and control groups according to time on renal replacement therapy (RRT) (a) and according to chronological age (b)

See this image and copyright information in PMC

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

Affiliations

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

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Abstract

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