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 Haffner & 102 others Anna 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

Fingerprint

Autosomal Recessive Polycystic Kidney
Nephrology
Liver Transplantation
Kidney Transplantation
Registries
Renal Dialysis
Pediatrics
Transplants
Kidney
Transplantation
Young Adult
Liver
Survival
Liver Cirrhosis
Observational Studies
Renal Insufficiency
Liver Diseases
Dialysis
Cohort Studies

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

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

In: American Journal of Kidney Diseases, Vol. 68, No. 5, 01.11.2016, p. 782-788.

Research output: Contribution to journalArticle

Mekahli, D, van Stralen, KJ, Bonthuis, M, Jager, KJ, Balat, A, Benetti, E, Godefroid, N, Edvardsson, VO, Heaf, JG, Jankauskiene, A, Kerecuk, L, Marinova, S, Puteo, F, Seeman, T, Zurowska, A, Pirenne, J, Schaefer, F, Groothoff, JW, Levtchenko, EN, Haffner, D, Bjerre, A, Massy, Z, Shtiza, D, Kramar, R, Oberbauer, R, Baiko, S, Sukalo, A, Van Hoeck, K, Collart, F, des Grottes, JM, Pokrajac, D, Roussinov, DL, Batinić, D, Lemac, M, Slavicek, J, Seeman, T, Vondrak, K, Heaf, JG, Toots, U, Finne, P, Gronhagen-Riska, C, Couchoud, C, Lasalle, M, Sahpazova, E, Abazi, N, Ristoka Bojkovska, N, von Gersdorff, G, Scholz, C, Tönshoff, B, Krupka, K, Höcker, B, Pape, L, Afentakis, N, Kapogiannis, A, Printza, N, Reusz, G, Berecki, C, Szabó, A, Szabó, T, Györke, ZS, Kis, E, Palsson, R, Edvardsson, V, Gianoglio, B, Maringhini, S, Pecoraro, C, Picca, S, Testa, S, Vidal, E, Verrina, E, Jankauskiené, A, Pundziene, B, Said-Conti, V, Gatcan, S, Berbeca, O, Zaikova, N, Pavićević, S, Leivestad, T, Zurowska, A, Zagozdzon, I, Mota, C, Almeida, M, Afonso, C, Mircescu, G, Garneata, L, Molchanova, EA, Tomilina, NA, Bikbov, B, Kostic, M, Peco-Antic, A, Spasojević-Dimitrijeva, B, Milosevski-Lomic, G, Paripović, D, Puric, S, Kruscic, D, Podracka, L, Kolvek, G, Buturovic-Ponikvar, J, Novljan, G, Battelino, N, Alonso Melgar, A, Schön, S, Prütz, KG, Bäckman, L, Stendahl, M, Evans, M, Rippe, B, Kuenhi, CE, Maurer, E, Laube, G, Tschumi, S, Parvex, P, Hoitsma, AJ, Hemke, A, Topaloglu, R, Duzova, A, Ivanov, D, Pruthi, R, Braddon, F, Mannings, S, Cassula, A & Sinha, MD 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, vol. 68, no. 5, pp. 782-788. https://doi.org/10.1053/j.ajkd.2016.06.019
Mekahli, Djalila ; van Stralen, Karlijn J. ; Bonthuis, Marjolein ; Jager, Kitty J. ; Balat, Ayse ; Benetti, Elisa ; Godefroid, Nathalie ; Edvardsson, Vidar O. ; Heaf, James G. ; Jankauskiene, Augustina ; Kerecuk, Larissa ; Marinova, Svetlana ; Puteo, Flora ; Seeman, Tomas ; Zurowska, Aleksandra ; Pirenne, J. ; Schaefer, F. ; Groothoff, Jaap W. ; Levtchenko, Elena N. ; Haffner, Dieter ; Bjerre, Anna ; Massy, Ziad ; Shtiza, D. ; Kramar, R. ; Oberbauer, R. ; Baiko, Sergey ; Sukalo, A. ; Van Hoeck, Koen ; Collart, Frederic ; des Grottes, J. M. ; Pokrajac, D. ; Roussinov, Dimitar L. ; Batinić, D. ; Lemac, M. ; Slavicek, J. ; Seeman, T. ; Vondrak, Karel ; Heaf, James G. ; Toots, U. ; Finne, Patrik ; Gronhagen-Riska, C. ; Couchoud, C. ; Lasalle, M. ; Sahpazova, Emilija ; Abazi, N. ; Ristoka Bojkovska, N. ; von Gersdorff, G. ; Scholz, C. ; Tönshoff, B. ; Krupka, K. ; Höcker, B. ; Pape, Lars ; Afentakis, N. ; Kapogiannis, A. ; Printza, Nikoleta ; Reusz, György ; Berecki, Cs ; Szabó, A. ; Szabó, T. ; Györke, Z. S. ; Kis, Eva ; Palsson, Runolfur ; Edvardsson, V. ; Gianoglio, Bruno ; Maringhini, Silvio ; Pecoraro, Carmine ; Picca, Stefano ; Testa, Sara ; Vidal, E. ; Verrina, Enrico ; Jankauskiené, A. ; Pundziene, Birute ; Said-Conti, V. ; Gatcan, S. ; Berbeca, O. ; Zaikova, N. ; Pavićević, S. ; Leivestad, Torbjørn ; Zurowska, Aleksandra ; Zagozdzon, Ilona ; Mota, C. ; Almeida, M. ; Afonso, C. ; Mircescu, G. ; Garneata, L. ; Molchanova, Elena A. ; Tomilina, N. A. ; Bikbov, Boris ; Kostic, M. ; Peco-Antic, Amira ; Spasojević-Dimitrijeva, Brankica ; Milosevski-Lomic, G. ; Paripović, Dušan ; Puric, S. ; Kruscic, D. ; Podracka, Ludmila ; Kolvek, G. ; Buturovic-Ponikvar, J. ; Novljan, Gregor ; Battelino, Nina ; Alonso Melgar, A. ; Schön, S. ; Prütz, K. G. ; Bäckman, Lars ; Stendahl, M. ; Evans, M. ; Rippe, B. ; Kuenhi, C. E. ; Maurer, E. ; Laube, G. ; Tschumi, S. ; Parvex, Paloma ; Hoitsma, Andries J. ; Hemke, A. ; Topaloglu, Rezan ; Duzova, Ali ; Ivanov, Dimitar ; Pruthi, R. ; Braddon, F. ; Mannings, S. ; Cassula, A. ; Sinha, Manish D. / 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. In: American Journal of Kidney Diseases. 2016 ; Vol. 68, No. 5. pp. 782-788.
@article{5f3566a2b76a4c889ef925a3ddd47f71,
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. 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.",
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",
author = "Djalila Mekahli and {van Stralen}, {Karlijn J.} and Marjolein Bonthuis and Jager, {Kitty J.} and Ayse Balat and Elisa Benetti and Nathalie Godefroid and Edvardsson, {Vidar O.} and Heaf, {James G.} and Augustina Jankauskiene and Larissa Kerecuk and Svetlana Marinova and Flora Puteo and Tomas Seeman and Aleksandra Zurowska and J. Pirenne and F. Schaefer and Groothoff, {Jaap W.} and Levtchenko, {Elena N.} and Dieter Haffner and Anna Bjerre and Ziad Massy and D. Shtiza and R. Kramar and R. Oberbauer and Sergey Baiko and A. Sukalo and {Van Hoeck}, Koen and Frederic Collart and {des Grottes}, {J. M.} and D. Pokrajac and Roussinov, {Dimitar L.} and D. Batinić and M. Lemac and J. Slavicek and T. Seeman and Karel Vondrak and Heaf, {James G.} and U. Toots and Patrik Finne and C. Gronhagen-Riska and C. Couchoud and M. Lasalle and Emilija Sahpazova and N. Abazi and {Ristoka Bojkovska}, N. and {von Gersdorff}, G. and C. Scholz and B. T{\"o}nshoff and K. Krupka and B. H{\"o}cker and Lars Pape and N. Afentakis and A. Kapogiannis and Nikoleta Printza and Gy{\"o}rgy Reusz and Cs Berecki and A. Szab{\'o} and T. Szab{\'o} and Gy{\"o}rke, {Z. S.} and Eva Kis and Runolfur Palsson and V. Edvardsson and Bruno Gianoglio and Silvio Maringhini and Carmine Pecoraro and Stefano Picca and Sara Testa and E. Vidal and Enrico Verrina and A. Jankauskien{\'e} and Birute Pundziene and V. Said-Conti and S. Gatcan and O. Berbeca and N. Zaikova and S. Pavićević and Torbj{\o}rn Leivestad and Aleksandra Zurowska and Ilona Zagozdzon and C. Mota and M. Almeida and C. Afonso and G. Mircescu and L. Garneata and Molchanova, {Elena A.} and Tomilina, {N. A.} and Boris Bikbov and M. Kostic and Amira Peco-Antic and Brankica Spasojević-Dimitrijeva and G. Milosevski-Lomic and Dušan Paripović and S. Puric and D. Kruscic and Ludmila Podracka and G. Kolvek and J. Buturovic-Ponikvar and Gregor Novljan and Nina Battelino and {Alonso Melgar}, A. and S. Sch{\"o}n and Pr{\"u}tz, {K. G.} and Lars B{\"a}ckman and M. Stendahl and M. Evans and B. Rippe and Kuenhi, {C. E.} and E. Maurer and G. Laube and S. Tschumi and Paloma Parvex and Hoitsma, {Andries J.} and A. Hemke and Rezan Topaloglu and Ali Duzova and Dimitar Ivanov and R. Pruthi and F. Braddon and S. Mannings and A. Cassula and Sinha, {Manish D.}",
year = "2016",
month = "11",
day = "1",
doi = "10.1053/j.ajkd.2016.06.019",
language = "English",
volume = "68",
pages = "782--788",
journal = "American Journal of Kidney Diseases",
issn = "0272-6386",
publisher = "W.B. Saunders Ltd",
number = "5",

}

TY - JOUR

T1 - Kidney Versus Combined Kidney and Liver Transplantation in Young People With Autosomal Recessive Polycystic Kidney Disease

T2 - Data From the European Society for Pediatric Nephrology/European Renal Association−European Dialysis and Transplant (ESPN/ERA-EDTA) Registry

AU - Mekahli, Djalila

AU - van Stralen, Karlijn J.

AU - Bonthuis, Marjolein

AU - Jager, Kitty J.

AU - Balat, Ayse

AU - Benetti, Elisa

AU - Godefroid, Nathalie

AU - Edvardsson, Vidar O.

AU - Heaf, James G.

AU - Jankauskiene, Augustina

AU - Kerecuk, Larissa

AU - Marinova, Svetlana

AU - Puteo, Flora

AU - Seeman, Tomas

AU - Zurowska, Aleksandra

AU - Pirenne, J.

AU - Schaefer, F.

AU - Groothoff, Jaap W.

AU - Levtchenko, Elena N.

AU - Haffner, Dieter

AU - Bjerre, Anna

AU - Massy, Ziad

AU - Shtiza, D.

AU - Kramar, R.

AU - Oberbauer, R.

AU - Baiko, Sergey

AU - Sukalo, A.

AU - Van Hoeck, Koen

AU - Collart, Frederic

AU - des Grottes, J. M.

AU - Pokrajac, D.

AU - Roussinov, Dimitar L.

AU - Batinić, D.

AU - Lemac, M.

AU - Slavicek, J.

AU - Seeman, T.

AU - Vondrak, Karel

AU - Heaf, James G.

AU - Toots, U.

AU - Finne, Patrik

AU - Gronhagen-Riska, C.

AU - Couchoud, C.

AU - Lasalle, M.

AU - Sahpazova, Emilija

AU - Abazi, N.

AU - Ristoka Bojkovska, N.

AU - von Gersdorff, G.

AU - Scholz, C.

AU - Tönshoff, B.

AU - Krupka, K.

AU - Höcker, B.

AU - Pape, Lars

AU - Afentakis, N.

AU - Kapogiannis, A.

AU - Printza, Nikoleta

AU - Reusz, György

AU - Berecki, Cs

AU - Szabó, A.

AU - Szabó, T.

AU - Györke, Z. S.

AU - Kis, Eva

AU - Palsson, Runolfur

AU - Edvardsson, V.

AU - Gianoglio, Bruno

AU - Maringhini, Silvio

AU - Pecoraro, Carmine

AU - Picca, Stefano

AU - Testa, Sara

AU - Vidal, E.

AU - Verrina, Enrico

AU - Jankauskiené, A.

AU - Pundziene, Birute

AU - Said-Conti, V.

AU - Gatcan, S.

AU - Berbeca, O.

AU - Zaikova, N.

AU - Pavićević, S.

AU - Leivestad, Torbjørn

AU - Zurowska, Aleksandra

AU - Zagozdzon, Ilona

AU - Mota, C.

AU - Almeida, M.

AU - Afonso, C.

AU - Mircescu, G.

AU - Garneata, L.

AU - Molchanova, Elena A.

AU - Tomilina, N. A.

AU - Bikbov, Boris

AU - Kostic, M.

AU - Peco-Antic, Amira

AU - Spasojević-Dimitrijeva, Brankica

AU - Milosevski-Lomic, G.

AU - Paripović, Dušan

AU - Puric, S.

AU - Kruscic, D.

AU - Podracka, Ludmila

AU - Kolvek, G.

AU - Buturovic-Ponikvar, J.

AU - Novljan, Gregor

AU - Battelino, Nina

AU - Alonso Melgar, A.

AU - Schön, S.

AU - Prütz, K. G.

AU - Bäckman, Lars

AU - Stendahl, M.

AU - Evans, M.

AU - Rippe, B.

AU - Kuenhi, C. E.

AU - Maurer, E.

AU - Laube, G.

AU - Tschumi, S.

AU - Parvex, Paloma

AU - Hoitsma, Andries J.

AU - Hemke, A.

AU - Topaloglu, Rezan

AU - Duzova, Ali

AU - Ivanov, Dimitar

AU - Pruthi, R.

AU - Braddon, F.

AU - Mannings, S.

AU - Cassula, A.

AU - Sinha, Manish D.

PY - 2016/11/1

Y1 - 2016/11/1

N2 - 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.

AB - 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.

KW - adolescents

KW - allograft survival

KW - Autosomal recessive polycystic kidney disease (ARPKD)

KW - children

KW - combined liver-kidney transplantation

KW - ESPN/ERA-EDTA Registry

KW - kidney transplantation

KW - mortality

KW - patient survival

KW - pediatric

KW - renal replacement therapy

KW - young adults

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U2 - 10.1053/j.ajkd.2016.06.019

DO - 10.1053/j.ajkd.2016.06.019

M3 - Article

VL - 68

SP - 782

EP - 788

JO - American Journal of Kidney Diseases

JF - American Journal of Kidney Diseases

SN - 0272-6386

IS - 5

ER -