title: Kidney 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.
abstract: BACKGROUND
  The 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.
  
  
  METHODS
  Cohort study.
  
  
  METHODS
  We 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.
  
  
  METHODS
  Liver transplantation.
  
  
  METHODS
  Transplantation and patient survival.
  
  
  RESULTS
  202 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).
  
  
  CONCLUSIONS
  No data for liver disease of kidney therapy recipients.
  
  
  CONCLUSIONS
  Combined 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.
authors.affiliation: Department of Pediatric Nephrology, University Hospitals of Leuven, Leuven, Belgium; Department of Development and Regeneration, KU Leuven, Leuven, Belgium.
authors.forename: Djalila
authors.initials: D
authors.lastname: Mekahli
authors.affiliation: ESPN/ERA-EDTA Registry, Department of Medical informatics, Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands.
authors.forename: Karlijn J
authors.initials: KJ
authors.lastname: van Stralen
authors.affiliation: ESPN/ERA-EDTA Registry, Department of Medical informatics, Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands. Electronic address: m.bonthuis@amc.uva.nl.
authors.forename: Marjolein
authors.initials: M
authors.lastname: Bonthuis
authors.affiliation: ESPN/ERA-EDTA Registry, Department of Medical informatics, Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands.
authors.forename: Kitty J
authors.initials: KJ
authors.lastname: Jager
authors.affiliation: Department of Pediatric Nephrology and Rheumatology, School of Medicine, Gaziantep University, Gaziantep, Turkey.
authors.forename: Ayşe
authors.initials: A
authors.lastname: Balat
authors.affiliation: Pediatric Nephrology, Dialysis and Transplant Unit, Department of Pediatrics, University of Padova, Italy.
authors.forename: Elisa
authors.initials: E
authors.lastname: Benetti
authors.affiliation: Department of Pediatrics, Université catholique de Louvain Medical School, Saint-Luc Academic Hospital, Brussels, Belgium.
authors.forename: Nathalie
authors.initials: N
authors.lastname: Godefroid
authors.affiliation: Faculty of Medicine, School of Health Sciences, University of Iceland, Reykjavik, Iceland; Children's Medical Center, Landspitali-The National University Hospital of Iceland, Iceland.
authors.forename: Vidar O
authors.initials: VO
authors.lastname: Edvardsson
authors.affiliation: Department of Medicine, Roskilde Hospital, University of Copenhagen, Copenhagen, Denmark.
authors.forename: James G
authors.initials: JG
authors.lastname: Heaf
authors.affiliation: Vilnius University Hospital, Center for Pediatrics, Vilnius, Lithuania.
authors.forename: Augustina
authors.initials: A
authors.lastname: Jankauskiene
authors.affiliation: Department of Pediatric Nephrology, Birmingham Children's Hospital, National Health Service Foundation Trust, Birmingham, United Kingdom.
authors.forename: Larissa
authors.initials: L
authors.lastname: Kerecuk
authors.affiliation: Clinic of Pediatric Nephrology and Dialysis, University of Sofia, Sofia, Bulgaria.
authors.forename: Svetlana
authors.initials: S
authors.lastname: Marinova
authors.affiliation: Pediatric Nephrology and Dialysis Unit, Pediatric Hospital Giovanni XXIII, Bari, Italy.
authors.forename: Flora
authors.initials: F
authors.lastname: Puteo
authors.affiliation: University Hospital Motol, 2nd School of Medicine, Charles University Prague, Prague, Czech Republic.
authors.forename: Tomas
authors.initials: T
authors.lastname: Seeman
authors.affiliation: Department for Pediatrics, Nephrology & Hypertension, Medical University of Gdansk, Gdansk, Poland.
authors.forename: Aleksandra
authors.initials: A
authors.lastname: Zurowska
authors.affiliation: Department of Abdominal Transplantation, University Hospitals of Leuven, Leuven, Belgium.
authors.forename: Jacques
authors.initials: J
authors.lastname: Pirenne
authors.affiliation: University of Heidelberg Center for Pediatrics and Adolescent Medicine, Heidelberg, Germany.
authors.forename: Franz
authors.initials: F
authors.lastname: Schaefer
authors.affiliation: Department of Pediatric, Academic Medical Center, Amsterdam, the Netherlands.
authors.forename: Jaap W
authors.initials: JW
authors.lastname: Groothoff
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D005260:Female; D006801:Humans; D007223:Infant; D016030:Kidney Transplantation; D008103:Liver Cirrhosis / Q000209:etiology* / Q000401:mortality / Q000601:surgery*; D016031:Liver Transplantation; D008297:Male; D017044:Polycystic Kidney, Autosomal Recessive / Q000150:complications* / Q000401:mortality; D012042:Registries; D051437:Renal Insufficiency / Q000209:etiology* / Q000401:mortality / Q000601:surgery*; D012955:Societies, Medical; D016019:Survival Analysis
keywords: Autosomal recessive polycystic kidney disease (ARPKD); ESPN/ERA-EDTA Registry; adolescents; allograft survival; children; combined liver-kidney transplantation; kidney transplantation; mortality; patient survival; pediatric; renal replacement therapy; young adults