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

Djalila Mekahli, Karlijn J. van Stralen, Marjolein Bonthuis, Kitty J. Jager, Ayse Balat, Elisa Benetti, Nathalie Godefroid, Vidar O. Edvardsson, James G. Heaf, Augustina Jankauskiene, Larissa Kerecuk, Svetlana Marinova, Flora Puteo, Tomas Seeman, Aleksandra Zurowska, J. Pirenne, F. Schaefer, Jaap W. Groothoff, Elena N. Levtchenko, Dieter HaffnerAnna Bjerre, Ziad Massy, D. Shtiza, R. Kramar, R. Oberbauer, Sergey Baiko, A. Sukalo, Koen Van Hoeck, Frederic Collart, J. M. des Grottes, D. Pokrajac, Dimitar L. Roussinov, D. Batinić, M. Lemac, J. Slavicek, T. Seeman, Karel Vondrak, James G. Heaf, U. Toots, Patrik Finne, C. Gronhagen-Riska, C. Couchoud, M. Lasalle, Emilija Sahpazova, N. Abazi, N. Ristoka Bojkovska, G. von Gersdorff, C. Scholz, B. Tönshoff, K. Krupka, B. Höcker, Lars Pape, N. Afentakis, A. Kapogiannis, Nikoleta Printza, György Reusz, Cs Berecki, A. Szabó, T. Szabó, Z. S. Györke, Eva Kis, Runolfur Palsson, V. Edvardsson, Bruno Gianoglio, Silvio Maringhini, Carmine Pecoraro, Stefano Picca, Sara Testa, E. Vidal, Enrico Verrina, A. Jankauskiené, Birute Pundziene, V. Said-Conti, S. Gatcan, O. Berbeca, N. Zaikova, S. Pavićević, Torbjørn Leivestad, Aleksandra Zurowska, Ilona Zagozdzon, C. Mota, M. Almeida, C. Afonso, G. Mircescu, L. Garneata, Elena A. Molchanova, N. A. Tomilina, Boris Bikbov, M. Kostic, Amira Peco-Antic, Brankica Spasojević-Dimitrijeva, G. Milosevski-Lomic, Dušan Paripović, S. Puric, D. Kruscic, Ludmila Podracka, G. Kolvek, J. Buturovic-Ponikvar, Gregor Novljan, Nina Battelino, A. Alonso Melgar, S. Schön, K. G. Prütz, Lars Bäckman, M. Stendahl, M. Evans, B. Rippe, C. E. Kuenhi, E. Maurer, G. Laube, S. Tschumi, Paloma Parvex, Andries J. Hoitsma, A. Hemke, Rezan Topaloglu, Ali Duzova, Dimitar Ivanov, R. Pruthi, F. Braddon, S. Mannings, A. Cassula, Manish D. Sinha

Research output: Contribution to journalArticle

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. 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.

Original languageEnglish
Pages (from-to)782-788
Number of pages7
JournalAmerican Journal of Kidney Diseases
Volume68
Issue number5
DOIs
Publication statusPublished - Nov 1 2016

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Keywords

  • adolescents
  • allograft survival
  • Autosomal recessive polycystic kidney disease (ARPKD)
  • children
  • combined liver-kidney transplantation
  • ESPN/ERA-EDTA Registry
  • kidney transplantation
  • mortality
  • patient survival
  • pediatric
  • renal replacement therapy
  • young adults

ASJC Scopus subject areas

  • Nephrology

Cite this

Mekahli, D., van Stralen, K. J., Bonthuis, M., Jager, K. J., Balat, A., Benetti, E., Godefroid, N., Edvardsson, V. O., Heaf, J. G., Jankauskiene, A., Kerecuk, L., Marinova, S., Puteo, F., Seeman, T., Zurowska, A., Pirenne, J., Schaefer, F., Groothoff, J. W., Levtchenko, E. N., ... Sinha, M. D. (2016). 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. American Journal of Kidney Diseases, 68(5), 782-788. https://doi.org/10.1053/j.ajkd.2016.06.019