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, Ayşe Balat, Elisa Benetti, Nathalie Godefroid, Vidar O. Edvardsson, James G. Heaf, Augustina Jankauskiene, Larissa Kerecuk, Svetlana Marinova, Flora Puteo, Tomas Seeman, Aleksandra Zurowska, Jacques Pirenne, Franz Schaefer, Jaap W. Groothoff, E. Levtchenko, D. HaffnerA. Bjerre, Z. Massy, D. Shtiza, R. Kramar, R. Oberbauer, S. Baiko, A. Sukalo, K. van Hoeck, F. Collart, J. M. des Grottes, D. Pokrajac, D. Roussinov, D. Batinić, M. Lemac, J. Slavicek, T. Seeman, K. Vondrak, J. G. Heaf, U. Toots, P. Finne, C. Grönhagen-Riska, C. Couchoud, M. Lasalle, E. Sahpazova, N. Abazi, N. Ristoka Bojkovska, G. von Gersdorff, C. Scholz, B. Tönshoff, K. Krupka, B. Höcker, L. Pape, N. Afentakis, A. Kapogiannis, N. Printza, G. Reusz, Cs Berecki, A. Szabó, T. Szabó, Z. S. Györke, E. Kis, R. Palsson, V. Edvardsson, B. Gianoglio, S. Maringhini, C. Pecoraro, S. Picca, S. Testa, E. Vidal, E. Verrina, A. Jankauskiene, B. Pundziene, V. Said-Conti, S. Gatcan, O. Berbeca, N. Zaikova, S. Pavićević, T. Leivestad, A. Zurowska, I. Zagozdzon, C. Mota, M. Almeida, C. Afonso, G. Mircescu, L. Garneata, E. A. Molchanova, N. A. Tomilina, B. T. Bikbov, M. Kostic, A. Peco-Antic, B. Spasojevic-Dimitrijeva, G. Milosevski-Lomic, D. Paripovic, S. Puric, D. Kruscic, L. Podracka, G. Kolvek, J. Buturovic-Ponikvar, G. Novljan, N. Battelino, A. Alonso Melgar, S. Schön, K. G. Prütz, L. Backmän, M. Stendahl, M. Evans, B. Rippe, C. E. Kuenhi, E. Maurer, G. F. Laube, S. Tschumi, P. Parvex, A. Hoitsma, A. Hemke, R. Topaloglu, A. Duzova, D. Ivanov, R. Pruthi, F. Braddon, S. Mannings, A. Cassula, M. 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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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, Ayşe; Benetti, Elisa; Godefroid, Nathalie; Edvardsson, Vidar O.; Heaf, James G.; Jankauskiene, Augustina; Kerecuk, Larissa; Marinova, Svetlana; Puteo, Flora; Seeman, Tomas; Zurowska, Aleksandra; Pirenne, Jacques; Schaefer, Franz; Groothoff, Jaap W.; Levtchenko, E.; Haffner, D.; Bjerre, A.; Massy, Z.; Shtiza, D.; Kramar, R.; Oberbauer, R.; Baiko, S.; Sukalo, A.; van Hoeck, K.; Collart, F.; des Grottes, J. M.; Pokrajac, D.; Roussinov, D.; Batinić, D.; Lemac, M.; Slavicek, J.; Seeman, T.; Vondrak, K.; Heaf, J. G.; Toots, U.; Finne, P.; Grönhagen-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, Cs; Szabó, A.; Szabó, T.; Györke, Z. S.; Kis, E.; Palsson, R.; Edvardsson, V.; Gianoglio, B.; Maringhini, S.; Pecoraro, C.; Picca, S.; Testa, S.; Vidal, E.; Verrina, E.; Jankauskiene, 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, E. A.; Tomilina, N. A.; Bikbov, B. T.; Kostic, M.; Peco-Antic, A.; Spasojevic-Dimitrijeva, B.; Milosevski-Lomic, G.; Paripovic, D.; Puric, S.; Kruscic, D.; Podracka, L.; Kolvek, G.; Buturovic-Ponikvar, J.; Novljan, G.; Battelino, N.; Alonso Melgar, A.; Schön, S.; Prütz, K. G.; Backmän, L.; Stendahl, M.; Evans, M.; Rippe, B.; Kuenhi, C. E.; Maurer, E.; Laube, G. F.; Tschumi, S.; Parvex, P.; Hoitsma, A.; Hemke, A.; Topaloglu, R.; Duzova, A.; Ivanov, D.; Pruthi, R.; Braddon, F.; Mannings, S.; Cassula, A.; Sinha, M. 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, E, 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, D, Batinić, D, Lemac, M, Slavicek, J, Seeman, T, Vondrak, K, Heaf, JG, Toots, U, Finne, P, Grönhagen-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, Jankauskiene, 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, BT, Kostic, M, Peco-Antic, A, Spasojevic-Dimitrijeva, B, Milosevski-Lomic, G, Paripovic, D, Puric, S, Kruscic, D, Podracka, L, Kolvek, G, Buturovic-Ponikvar, J, Novljan, G, Battelino, N, Alonso Melgar, A, Schön, S, Prütz, KG, Backmän, L, Stendahl, M, Evans, M, Rippe, B, Kuenhi, CE, Maurer, E, Laube, GF, Tschumi, S, Parvex, P, Hoitsma, A, 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, Ayşe ; Benetti, Elisa ; Godefroid, Nathalie ; Edvardsson, Vidar O. ; Heaf, James G. ; Jankauskiene, Augustina ; Kerecuk, Larissa ; Marinova, Svetlana ; Puteo, Flora ; Seeman, Tomas ; Zurowska, Aleksandra ; Pirenne, Jacques ; Schaefer, Franz ; Groothoff, Jaap W. ; Levtchenko, E. ; Haffner, D. ; Bjerre, A. ; Massy, Z. ; Shtiza, D. ; Kramar, R. ; Oberbauer, R. ; Baiko, S. ; Sukalo, A. ; van Hoeck, K. ; Collart, F. ; des Grottes, J. M. ; Pokrajac, D. ; Roussinov, D. ; Batinić, D. ; Lemac, M. ; Slavicek, J. ; Seeman, T. ; Vondrak, K. ; Heaf, J. G. ; Toots, U. ; Finne, P. ; Grönhagen-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, Cs ; Szabó, A. ; Szabó, T. ; Györke, Z. S. ; Kis, E. ; Palsson, R. ; Edvardsson, V. ; Gianoglio, B. ; Maringhini, S. ; Pecoraro, C. ; Picca, S. ; Testa, S. ; Vidal, E. ; Verrina, E. ; Jankauskiene, 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, E. A. ; Tomilina, N. A. ; Bikbov, B. T. ; Kostic, M. ; Peco-Antic, A. ; Spasojevic-Dimitrijeva, B. ; Milosevski-Lomic, G. ; Paripovic, D. ; Puric, S. ; Kruscic, D. ; Podracka, L. ; Kolvek, G. ; Buturovic-Ponikvar, J. ; Novljan, G. ; Battelino, N. ; Alonso Melgar, A. ; Schön, S. ; Prütz, K. G. ; Backmän, L. ; Stendahl, M. ; Evans, M. ; Rippe, B. ; Kuenhi, C. E. ; Maurer, E. ; Laube, G. F. ; Tschumi, S. ; Parvex, P. ; Hoitsma, A. ; Hemke, A. ; Topaloglu, R. ; Duzova, A. ; Ivanov, D. ; Pruthi, R. ; Braddon, F. ; Mannings, S. ; Cassula, A. ; Sinha, M. 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{7fead7cbdd894ff887855ccca54e4cb5,
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 Ayşe 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 Jacques Pirenne and Franz Schaefer and Groothoff, {Jaap W.} and E. Levtchenko and D. Haffner and A. Bjerre and Z. Massy and D. Shtiza and R. Kramar and R. Oberbauer and S. Baiko and A. Sukalo and {van Hoeck}, K. and F. Collart and {des Grottes}, {J. M.} and D. Pokrajac and D. Roussinov and D. Batinić and M. Lemac and J. Slavicek and T. Seeman and K. Vondrak and Heaf, {J. G.} and U. Toots and P. Finne and C. Gr{\"o}nhagen-Riska and C. Couchoud and M. Lasalle and E. 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 L. Pape and N. Afentakis and A. Kapogiannis and N. Printza and G. Reusz and Cs Berecki and A. Szab{\'o} and T. Szab{\'o} and Gy{\"o}rke, {Z. S.} and E. Kis and R. Palsson and V. Edvardsson and B. Gianoglio and S. Maringhini and C. Pecoraro and S. Picca and S. Testa and E. Vidal and E. Verrina and A. Jankauskiene and B. Pundziene and V. Said-Conti and S. Gatcan and O. Berbeca and N. Zaikova and S. Pavićević and T. Leivestad and A. Zurowska and I. Zagozdzon and C. Mota and M. Almeida and C. Afonso and G. Mircescu and L. Garneata and Molchanova, {E. A.} and Tomilina, {N. A.} and Bikbov, {B. T.} and M. Kostic and A. Peco-Antic and B. Spasojevic-Dimitrijeva and G. Milosevski-Lomic and D. Paripovic and S. Puric and D. Kruscic and L. Podracka and G. Kolvek and J. Buturovic-Ponikvar and G. Novljan and N. Battelino and {Alonso Melgar}, A. and S. Sch{\"o}n and Pr{\"u}tz, {K. G.} and L. Backm{\"a}n and M. Stendahl and M. Evans and B. Rippe and Kuenhi, {C. E.} and E. Maurer and Laube, {G. F.} and S. Tschumi and P. Parvex and A. Hoitsma and A. Hemke and R. Topaloglu and A. Duzova and D. Ivanov and R. Pruthi and F. Braddon and S. Mannings and A. Cassula and Sinha, {M. 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, Ayşe

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, Jacques

AU - Schaefer, Franz

AU - Groothoff, Jaap W.

AU - Levtchenko, E.

AU - Haffner, D.

AU - Bjerre, A.

AU - Massy, Z.

AU - Shtiza, D.

AU - Kramar, R.

AU - Oberbauer, R.

AU - Baiko, S.

AU - Sukalo, A.

AU - van Hoeck, K.

AU - Collart, F.

AU - des Grottes, J. M.

AU - Pokrajac, D.

AU - Roussinov, D.

AU - Batinić, D.

AU - Lemac, M.

AU - Slavicek, J.

AU - Seeman, T.

AU - Vondrak, K.

AU - Heaf, J. G.

AU - Toots, U.

AU - Finne, P.

AU - Grönhagen-Riska, C.

AU - Couchoud, C.

AU - Lasalle, M.

AU - Sahpazova, E.

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, L.

AU - Afentakis, N.

AU - Kapogiannis, A.

AU - Printza, N.

AU - Reusz, G.

AU - Berecki, Cs

AU - Szabó, A.

AU - Szabó, T.

AU - Györke, Z. S.

AU - Kis, E.

AU - Palsson, R.

AU - Edvardsson, V.

AU - Gianoglio, B.

AU - Maringhini, S.

AU - Pecoraro, C.

AU - Picca, S.

AU - Testa, S.

AU - Vidal, E.

AU - Verrina, E.

AU - Jankauskiene, A.

AU - Pundziene, B.

AU - Said-Conti, V.

AU - Gatcan, S.

AU - Berbeca, O.

AU - Zaikova, N.

AU - Pavićević, S.

AU - Leivestad, T.

AU - Zurowska, A.

AU - Zagozdzon, I.

AU - Mota, C.

AU - Almeida, M.

AU - Afonso, C.

AU - Mircescu, G.

AU - Garneata, L.

AU - Molchanova, E. A.

AU - Tomilina, N. A.

AU - Bikbov, B. T.

AU - Kostic, M.

AU - Peco-Antic, A.

AU - Spasojevic-Dimitrijeva, B.

AU - Milosevski-Lomic, G.

AU - Paripovic, D.

AU - Puric, S.

AU - Kruscic, D.

AU - Podracka, L.

AU - Kolvek, G.

AU - Buturovic-Ponikvar, J.

AU - Novljan, G.

AU - Battelino, N.

AU - Alonso Melgar, A.

AU - Schön, S.

AU - Prütz, K. G.

AU - Backmän, L.

AU - Stendahl, M.

AU - Evans, M.

AU - Rippe, B.

AU - Kuenhi, C. E.

AU - Maurer, E.

AU - Laube, G. F.

AU - Tschumi, S.

AU - Parvex, P.

AU - Hoitsma, A.

AU - Hemke, A.

AU - Topaloglu, R.

AU - Duzova, A.

AU - Ivanov, D.

AU - Pruthi, R.

AU - Braddon, F.

AU - Mannings, S.

AU - Cassula, A.

AU - Sinha, M. 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

AN - SCOPUS:84994887823

VL - 68

SP - 782

EP - 788

JO - American Journal of Kidney Diseases

JF - American Journal of Kidney Diseases

SN - 0272-6386

IS - 5

ER -