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

• Published in: American journal of kidney diseases Vol. 68; no. 5; pp. 782 - 788
• Main Authors: Mekahli, Djalila, MD, PhD, van Stralen, Karlijn J., PhD, Bonthuis, Marjolein, PhD, Jager, Kitty J., MD, PhD, Balat, Ayşe, MD, Benetti, Elisa, MD, Godefroid, Nathalie, MD, Edvardsson, Vidar O., MD, Heaf, James G., MD, DMSc, Jankauskiene, Augustina, MD, Kerecuk, Larissa, MBBS, BSc, MRCPCH, FRCPCH, Marinova, Svetlana, MD, Puteo, Flora, MD, Seeman, Tomas, MD, PhD, Zurowska, Aleksandra, MD, PhD, Pirenne, Jacques, MD, PhD, Schaefer, Franz, MD, Groothoff, Jaap W., MD, PhD
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 01.11.2016
• Subjects: Adolescent; adolescents; allograft survival; Autosomal recessive polycystic kidney disease (ARPKD); Child; Child, Preschool; children; Cohort Studies; combined liver-kidney transplantation; ESPN/ERA-EDTA Registry; Female; Humans; Infant; Kidney Transplantation; Liver Cirrhosis - etiology; Liver Cirrhosis - mortality; Liver Cirrhosis - surgery; Liver Transplantation; Male; mortality; Nephrology; patient survival; pediatric; Polycystic Kidney, Autosomal Recessive - complications; Polycystic Kidney, Autosomal Recessive - mortality; Registries; Renal Insufficiency - etiology; Renal Insufficiency - mortality; Renal Insufficiency - surgery; renal replacement therapy; Societies, Medical; Survival Analysis; young adults; renal replacement therapy; patient survival; children; adolescents; young adults; kidney transplantation; allograft survival; ESPN/ERA-EDTA Registry; mortality; Autosomal recessive polycystic kidney disease (ARPKD); combined liver-kidney transplantation; pediatric
• ISSN: 0272-6386; 1523-6838
• DOI: 10.1053/j.ajkd.2016.06.019

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.