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dc.contributor.authorMekahli, Djalila
dc.contributor.authorvan Stralen, Karlijn J
dc.contributor.authorBonthuis, Marjolein
dc.contributor.authorJager, Kitty J
dc.contributor.authorBalat, Ayşe
dc.contributor.authorBenetti, Elisa
dc.contributor.authorGodefroid, Nathalie
dc.contributor.authorEdvardsson, Vidar O
dc.contributor.authorHeaf, James G
dc.contributor.authorJankauskiene, Augustina
dc.contributor.authorKerecuk, Larissa
dc.contributor.authorMarinova, Svetlana
dc.contributor.authorPuteo, Flora
dc.contributor.authorSeeman, Tomas
dc.contributor.authorZurowska, Aleksandra
dc.contributor.authorPirenne, Jacques
dc.contributor.authorSchaefer, Franz
dc.contributor.authorGroothoff, Jaap W
dc.date.accessioned2016-08-29T11:11:31Z
dc.date.available2016-08-29T11:11:31Z
dc.date.issued2016-08-20
dc.date.submitted2016
dc.identifier.citationKidney Versus Combined Kidney and Liver Transplantation in Young People With Autosomal Recessive Polycystic Kidney Disease: Data From the European Society for Pediatric Nephrology/European Renal Association-European Dialysis and Transplant (ESPN/ERA-EDTA) Registry. Am J Kidney Dis. 2016 Nov;68(5):782-788en
dc.identifier.issn1523-6838
dc.identifier.pmid27555106
dc.identifier.doi10.1053/j.ajkd.2016.06.019
dc.identifier.urihttp://hdl.handle.net/2336/619019
dc.descriptionTo access publisher's full text version of this article click on the hyperlink at the bottom of the pageen
dc.description.abstractThe choice for either kidney or combined liver-kidney transplantation in young people with kidney failure and liver fibrosis due to autosomal recessive polycystic kidney disease (ARPKD) can be challenging. We aimed to analyze the characteristics and outcomes of transplantation type in these children, adolescents, and young adults.
dc.description.abstractCohort study.
dc.description.abstractWe derived data for children, adolescents, and young adults with ARPKD with either kidney or combined liver-kidney transplants for 1995 to 2012 from the ESPN/ERA-EDTA Registry, a European pediatric renal registry collecting data from 36 European countries.
dc.description.abstractLiver transplantation.
dc.description.abstractTransplantation and patient survival.
dc.description.abstract202 patients with ARPKD aged 19 years or younger underwent transplantation after a median of 0.4 (IQR, 0.0-1.4) years on dialysis therapy at a median age of 9.0 (IQR, 4.1-13.7) years. 32 (15.8%) underwent combined liver-kidney transplantation, 163 (80.7%) underwent kidney transplantation, and 7 (3.5%) were excluded because transplantation type was unknown. Age- and sex-adjusted 5-year patient survival posttransplantation was 95.5% (95% CI, 92.4%-98.8%) overall: 97.4% (95% CI, 94.9%-100.0%) for patients with kidney transplantation in contrast to 87.0% (95% CI, 75.8%-99.8%) with combined liver-kidney transplantation. The age- and sex-adjusted risk for death after combined liver-kidney transplantation was 6.7-fold (95% CI, 1.8- to 25.4-fold) greater than after kidney transplantation (P=0.005). Five-year death-censored kidney transplant survival following combined liver-kidney and kidney transplantation was similar (92.1% vs 85.9%; P=0.4).
dc.description.abstractNo data for liver disease of kidney therapy recipients.
dc.description.abstractCombined liver-kidney transplantation in ARPKD is associated with increased mortality compared to kidney transplantation in our large observational study and was not associated with improved 5-year kidney transplant survival. Long-term follow-up of both kidney and liver involvement are needed to better delineate the optimal transplantation strategy.
dc.description.sponsorshipESPN, the ERA-EDTA, and the European Commission. Clinical Research Fund of UZ Leuven. Rare Kidney Stone Consortium (U54KD083908), a part of the National Center for Advancing Translational Sciences (NCATS) Rare Diseases Clinical Research Network, which is an initiative of the Office of Rare Diseases Research. The Rare Kidney Stone Consortium is funded through collaboration between NCATS and National Institute of Diabetes and Digestive and Kidney Diseasesen
dc.languageENG
dc.language.isoenen
dc.publisherElsevieren
dc.relation.urlhttp://dx.doi.org/ 10.1053/j.ajkd.2016.06.019en
dc.rightsArchived with thanks to American journal of kidney diseases : the official journal of the National Kidney Foundationen
dc.subjectNýrnasjúkdómaren
dc.subjectBörnen
dc.subjectLíffæraflutningaren
dc.subjectLifrarígræðslaen
dc.subjectPED12
dc.subject.meshPolycystic Kidney, Autosomal Recessiveen
dc.subject.meshAdolescenten
dc.subject.meshChilden
dc.subject.meshChild, Preschoolen
dc.subject.meshAllograftsen
dc.subject.meshLiver Transplantationen
dc.subject.meshKidney Transplantationen
dc.subject.meshMortalityen
dc.subject.meshSurvivalen
dc.subject.meshRenal Replacement Therapyen
dc.titleKidney Versus Combined Kidney and Liver Transplantation in Young People With Autosomal Recessive Polycystic Kidney Disease: Data From the European Society for Pediatric Nephrology/European Renal Association-European Dialysis and Transplant (ESPN/ERA-EDTA) Registry.en
dc.typeArticleen
dc.contributor.departmentFrom the 1 Department of Pediatric Nephrology, University Hospitals of Leuven; 2 Department of Development and Regeneration, KU Leuven, Leuven, Belgium; 3 ESPN/ERA-EDTA Registry, Department of Medical informatics, Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands; 4 Department of Pediatric Nephrology and Rheumatology, School of Medicine, Gaziantep University, Gaziantep, Turkey; 5 Pediatric Nephrology, Dialysis and Transplant Unit, Department of Pediatrics, University of Padova, Italy; 6 Department of Pediatrics, Université catholique de Louvain Medical School, Saint-Luc Academic Hospital, Brussels, Belgium; 7 Faculty of Medicine, School of Health Sciences, University of Iceland, Reykjavik; 8 Children’s Medical Center, Landspitali-The National University Hospital of Iceland, Iceland; 9 Department of Medicine, Roskilde Hospital, University of Copenhagen, Copenhagen, Denmark; 10Vilnius University Hospital, Center for Pediatrics, Vilnius, Lithuania; 11Department of Pediatric Nephrology, Birmingham Children’s Hospital, National Health Service Foundation Trust, Birmingham, United Kingdom; 12Clinic of Pediatric Nephrology and Dialysis, University of Sofia, Sofia, Bulgaria; 13Pediatric Nephrology and Dialysis Unit, Pediatric Hospital Giovanni XXIII, Bari, Italy; 14University Hospital Motol, 2nd School of Medicine, Charles University Prague, Prague, Czech Republic; 15Department for Pediatrics, Nephrology & Hypertension, Medical University of Gdansk, Gdansk, Poland; 16Department of Abdominal Transplantation, University Hospitals of Leuven, Leuven, Belgium; 17University of Heidelberg Center for Pediatrics and Adolescent Medicine, Heidelberg, Germany; and 18Department of Pediatric, Academic Medical Center, Amsterdam, the Netherlands.en
dc.identifier.journalAmerican journal of kidney diseases : the official journal of the National Kidney Foundationen
dc.rights.accessNational Consortium - Landsaðganguren
html.description.abstractThe choice for either kidney or combined liver-kidney transplantation in young people with kidney failure and liver fibrosis due to autosomal recessive polycystic kidney disease (ARPKD) can be challenging. We aimed to analyze the characteristics and outcomes of transplantation type in these children, adolescents, and young adults.
html.description.abstractCohort study.
html.description.abstractWe derived data for children, adolescents, and young adults with ARPKD with either kidney or combined liver-kidney transplants for 1995 to 2012 from the ESPN/ERA-EDTA Registry, a European pediatric renal registry collecting data from 36 European countries.
html.description.abstractLiver transplantation.
html.description.abstractTransplantation and patient survival.
html.description.abstract202 patients with ARPKD aged 19 years or younger underwent transplantation after a median of 0.4 (IQR, 0.0-1.4) years on dialysis therapy at a median age of 9.0 (IQR, 4.1-13.7) years. 32 (15.8%) underwent combined liver-kidney transplantation, 163 (80.7%) underwent kidney transplantation, and 7 (3.5%) were excluded because transplantation type was unknown. Age- and sex-adjusted 5-year patient survival posttransplantation was 95.5% (95% CI, 92.4%-98.8%) overall: 97.4% (95% CI, 94.9%-100.0%) for patients with kidney transplantation in contrast to 87.0% (95% CI, 75.8%-99.8%) with combined liver-kidney transplantation. The age- and sex-adjusted risk for death after combined liver-kidney transplantation was 6.7-fold (95% CI, 1.8- to 25.4-fold) greater than after kidney transplantation (P=0.005). Five-year death-censored kidney transplant survival following combined liver-kidney and kidney transplantation was similar (92.1% vs 85.9%; P=0.4).
html.description.abstractNo data for liver disease of kidney therapy recipients.
html.description.abstractCombined liver-kidney transplantation in ARPKD is associated with increased mortality compared to kidney transplantation in our large observational study and was not associated with improved 5-year kidney transplant survival. Long-term follow-up of both kidney and liver involvement are needed to better delineate the optimal transplantation strategy.


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