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

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

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, 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

Department of Clinical Medicine

6 Citations (Scopus)

108 Downloads (Pure)

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.

Original language	English
Journal	Pediatric Nephrology
Volume	33
Issue number	1
Pages (from-to)	117-124
ISSN	0931-041X
DOIs	https://doi.org/10.1007/s00467-017-3770-9
Publication status	Published - 1 Jan 2018

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Journal Article

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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. (2018). Outcomes of renal replacement therapy in boys with prune belly syndrome: findings from the ESPN/ERA-EDTA Registry. Pediatric Nephrology, 33(1), 117-124. https://doi.org/10.1007/s00467-017-3770-9

Yalcinkaya, F, Bonthuis, M, Erdogan, BD, van Stralen, KJ, Baiko, S, Chehade, H, Maxwell, H, Montini, G, Rönnholm, K, Sørensen, SS, Ulinski, T, Verrina, E, Weber, S, Harambat, J, Schaefer, F, Jager, KJ & Groothoff, JW 2018, 'Outcomes of renal replacement therapy in boys with prune belly syndrome: findings from the ESPN/ERA-EDTA Registry', Pediatric Nephrology, vol. 33, no. 1, pp. 117-124. https://doi.org/10.1007/s00467-017-3770-9

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

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 = "Journal Article",

author = "Fatos Yalcinkaya and Marjolein Bonthuis and Erdogan, {Beyza Doganay} and {van Stralen}, {Karlijn J} and Sergey Baiko and Hassib Chehade and Heather Maxwell and Giovanni Montini and Kai R{\"o}nnholm and S{\o}rensen, {S{\o}ren Schwartz} and Tim Ulinski and Enrico Verrina and Stefanie Weber and J{\'e}r{\^o}me Harambat and Franz Schaefer and Jager, {Kitty J} and Groothoff, {Jaap W}",

year = "2018",

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day = "1",

doi = "10.1007/s00467-017-3770-9",

language = "English",

volume = "33",

pages = "117--124",

journal = "Pediatric Nephrology",

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TY - JOUR

T1 - Outcomes of renal replacement therapy in boys with prune belly syndrome

T2 - findings from the ESPN/ERA-EDTA Registry

AU - Yalcinkaya, Fatos

AU - Bonthuis, Marjolein

AU - Erdogan, Beyza Doganay

AU - van Stralen, Karlijn J

AU - Baiko, Sergey

AU - Chehade, Hassib

AU - Maxwell, Heather

AU - Montini, Giovanni

AU - Rönnholm, Kai

AU - Sørensen, Søren Schwartz

AU - Ulinski, Tim

AU - Verrina, Enrico

AU - Weber, Stefanie

AU - Harambat, Jérôme

AU - Schaefer, Franz

AU - Jager, Kitty J

AU - Groothoff, Jaap W

PY - 2018/1/1

Y1 - 2018/1/1

N2 - 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.

AB - 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.

KW - Journal Article

U2 - 10.1007/s00467-017-3770-9

DO - 10.1007/s00467-017-3770-9

M3 - Journal article

C2 - 28779237

SN - 0931-041X

VL - 33

SP - 117

EP - 124

JO - Pediatric Nephrology

JF - Pediatric Nephrology

IS - 1

ER -

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

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