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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
D. Mekahli, KJ. van Stralen, M. Bonthuis, KJ. Jager, A. Balat, E. Benetti, N. Godefroid, VO. Edvardsson, JG. Heaf, A. Jankauskiene, L. Kerecuk, S. Marinova, F. Puteo, T. Seeman, A. Zurowska, J. Pirenne, F. Schaefer, JW. Groothoff, . ,
Language English Country United States
Document type Comparative Study, Journal Article
- MeSH
- Survival Analysis MeSH
- Child MeSH
- Liver Cirrhosis etiology mortality surgery MeSH
- Cohort Studies MeSH
- Infant MeSH
- Humans MeSH
- Adolescent MeSH
- Polycystic Kidney, Autosomal Recessive complications mortality MeSH
- Child, Preschool MeSH
- Registries MeSH
- Renal Insufficiency etiology mortality surgery MeSH
- Societies, Medical MeSH
- Liver Transplantation * MeSH
- Kidney Transplantation * MeSH
- Check Tag
- Child MeSH
- Infant MeSH
- Humans MeSH
- Adolescent MeSH
- Male MeSH
- Child, Preschool MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
- Comparative Study MeSH
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. STUDY DESIGN: Cohort study. SETTING & PARTICIPANTS: 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. FACTOR: Liver transplantation. OUTCOMES & MEASUREMENTS: 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). LIMITATIONS: 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.
Children's Medical Center Landspitali The National University Hospital of Iceland Iceland
Clinic of Pediatric Nephrology and Dialysis University of Sofia Sofia Bulgaria
Department for Pediatrics Nephrology and Hypertension Medical University of Gdansk Gdansk Poland
Department of Abdominal Transplantation University Hospitals of Leuven Leuven Belgium
Department of Development and Regeneration KU Leuven Leuven Belgium
Department of Medicine Roskilde Hospital University of Copenhagen Copenhagen Denmark
Department of Pediatric Academic Medical Center Amsterdam the Netherlands
Department of Pediatric Nephrology University Hospitals of Leuven Leuven Belgium
Faculty of Medicine School of Health Sciences University of Iceland Reykjavik Iceland
Pediatric Nephrology and Dialysis Unit Pediatric Hospital Giovanni XXIII Bari Italy
University Hospital Motol 2nd School of Medicine Charles University Prague Prague Czech Republic
University of Heidelberg Center for Pediatrics and Adolescent Medicine Heidelberg Germany
Vilnius University Hospital Center for Pediatrics Vilnius Lithuania
References provided by Crossref.org
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- $a Mekahli, Djalila $u Department of Pediatric Nephrology, University Hospitals of Leuven, Leuven, Belgium; Department of Development and Regeneration, KU Leuven, Leuven, Belgium.
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- $a 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. STUDY DESIGN: Cohort study. SETTING & PARTICIPANTS: 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. FACTOR: Liver transplantation. OUTCOMES & MEASUREMENTS: 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). LIMITATIONS: 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.
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