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dc.rights.licenseopenen_US
dc.contributor.authorYALCINKAYA, F.
dc.contributor.authorBONTHUIS, M.
dc.contributor.authorERDOGAN, B. D.
dc.contributor.authorVAN STRALEN, K. J.
dc.contributor.authorBAIKO, S.
dc.contributor.authorCHEHADE, H.
dc.contributor.authorMAXWELL, H.
dc.contributor.authorMONTINI, G.
dc.contributor.authorRONNHOLM, K.
dc.contributor.authorSORENSEN, S. S.
dc.contributor.authorULINSKI, T.
dc.contributor.authorVERRINA, E.
dc.contributor.authorWEBER, S.
hal.structure.identifierBordeaux population health [BPH]
dc.contributor.authorHARAMBAT, Jerome
IDREF: 110567358
dc.contributor.authorSCHAEFER, F.
dc.contributor.authorJAGER, K. J.
dc.contributor.authorGROOTHOFF, J. W.
dc.date.accessioned2021-01-07T10:07:26Z
dc.date.available2021-01-07T10:07:26Z
dc.date.issued2018-01
dc.identifier.issn1432-198X (Electronic) 0931-041X (Linking)en_US
dc.identifier.urihttps://oskar-bordeaux.fr/handle/20.500.12278/23729
dc.description.abstractEnBACKGROUND: 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.
dc.language.isoENen_US
dc.rightsAttribution 3.0 United States*
dc.rights.urihttp://creativecommons.org/licenses/by/3.0/us/*
dc.subject.enLEHA
dc.title.enOutcomes of renal replacement therapy in boys with prune belly syndrome: findings from the ESPN/ERA-EDTA Registry
dc.title.alternativePediatr Nephrolen_US
dc.typeArticle de revueen_US
dc.identifier.doi10.1007/s00467-017-3770-9en_US
dc.subject.halSciences du Vivant [q-bio]/Santé publique et épidémiologieen_US
bordeaux.journalPediatric Nephrologyen_US
bordeaux.page117-124en_US
bordeaux.volume33en_US
bordeaux.hal.laboratoriesBordeaux Population Health Research Center (BPH) - UMR 1219en_US
bordeaux.issue1en_US
bordeaux.institutionUniversité de Bordeauxen_US
bordeaux.teamLEHA_BPH
bordeaux.teamLEHA_BPH
bordeaux.peerReviewedouien_US
bordeaux.inpressnonen_US
hal.identifierhal-03101597
hal.version1
hal.date.transferred2021-01-07T10:07:35Z
hal.exporttrue
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