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  • Background and objectives Primary hyperoxaluria (PH) as a cause of ESRD in children is believed to have poor outcomes. Data on management and outcomes of these children remain scarce. Design, setting, participants, & measurements This study included patients aged <19 years who started renal replacement therapy (RRT) between 1979 and 2009 from 31 countries providing data to a large European registry. Results Of 9247 incident patients receiving RRT, 100 patients had PH. PH children were significantly younger than non-PH children at the start of RRT. The median age at RRT of PH children decreased from 9.8 years in 1979-1989 to 1.5 years in 2000-2009. Survival was 86%, 79%, and 76% among PH patients at 1, 3, and 5 years after the start of RRT, compared with 97%, 94%, and 92% in non-PH patients, resulting in a three-fold increased risk of death over non-PH patients. PH and non-PH patient survival improved over time. Sixty-eight PH children received a first kidney (n=13) or liver-kidney transplantation (n=55). Although the comparison was hampered by the lower number of kidney transplantations primarily derived from the earlier era of RRT, kidney graft survival in PH patients was 82%, 79%, and 76% at 1, 3, and 5 years for liver-kidney transplantation and 46%, 28%, and 14% at 1, 3, and 5 years for kidney transplantation alone, compared with 95%, 90%, and 85% in non-PH patients. Conclusions The outcomes of PH children with ESRD are still poorer than in non-PH children but have substantially improved over time.
  • Background and objectives Primary hyperoxaluria (PH) as a cause of ESRD in children is believed to have poor outcomes. Data on management and outcomes of these children remain scarce. Design, setting, participants, & measurements This study included patients aged <19 years who started renal replacement therapy (RRT) between 1979 and 2009 from 31 countries providing data to a large European registry. Results Of 9247 incident patients receiving RRT, 100 patients had PH. PH children were significantly younger than non-PH children at the start of RRT. The median age at RRT of PH children decreased from 9.8 years in 1979-1989 to 1.5 years in 2000-2009. Survival was 86%, 79%, and 76% among PH patients at 1, 3, and 5 years after the start of RRT, compared with 97%, 94%, and 92% in non-PH patients, resulting in a three-fold increased risk of death over non-PH patients. PH and non-PH patient survival improved over time. Sixty-eight PH children received a first kidney (n=13) or liver-kidney transplantation (n=55). Although the comparison was hampered by the lower number of kidney transplantations primarily derived from the earlier era of RRT, kidney graft survival in PH patients was 82%, 79%, and 76% at 1, 3, and 5 years for liver-kidney transplantation and 46%, 28%, and 14% at 1, 3, and 5 years for kidney transplantation alone, compared with 95%, 90%, and 85% in non-PH patients. Conclusions The outcomes of PH children with ESRD are still poorer than in non-PH children but have substantially improved over time. (en)
Title
  • Characteristics and Outcomes of Children with Primary Oxalosis Requiring Renal Replacement Therapy
  • Characteristics and Outcomes of Children with Primary Oxalosis Requiring Renal Replacement Therapy (en)
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  • Characteristics and Outcomes of Children with Primary Oxalosis Requiring Renal Replacement Therapy
  • Characteristics and Outcomes of Children with Primary Oxalosis Requiring Renal Replacement Therapy (en)
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  • RIV/00064203:_____/12:8589!RIV13-MZ0-00064203
http://linked.open...avai/predkladatel
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  • 126780
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  • RIV/00064203:_____/12:8589
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  • primary hyperoxaluria type-1; liver-kidney transplantation; allograft survival; united-states; registry; growth; experience; mutation; infants; disease (en)
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  • US - Spojené státy americké
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  • Clinical Journal of the American Society of Nephrology
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  • 7
http://linked.open...iv/tvurceVysledku
  • Couchoud, C.
  • Schaefer, F.
  • Vondrák, Karel
  • Groothoff, JW
  • Jäger, KJ
  • Novljan, G.
  • Seeman, Tomáš
  • Verrina, E.
  • Leozappa, G.
  • Sahpazova, E.
  • Cochat, P.
  • Afonso, C.
  • Almeida, M.
  • Baiko, S.
  • Batinic, D.
  • Battelino, N.
  • Bikbov, BT
  • Brackman, D.
  • Buturovic-Ponikvar, J.
  • Cerkauskiene, R.
  • Collart, F.
  • Espinosa, L.
  • Finne, P.
  • Gafencu, M.
  • Garneata, L.
  • Gersdorf, G.
  • Gianoglio, B.
  • Grottes, JM
  • Grönhagen-Riska, C.
  • Harambat, J.
  • Heaf, J.
  • Hoeck, K.
  • Holmberg, C.
  • Hulton, S. A.
  • Ioannidis, G.
  • Jankauskiene, A.
  • Kolvek, G.
  • Kostic, M.
  • Kramar, R.
  • Leivestad, T.
  • Lombaerts, R.
  • Mencarelli, F.
  • Mignot, L.
  • Minale, B.
  • Mircescu, G.
  • Molchanova, E. A.
  • Mota, C.
  • Niaudet, P.
  • Oberbauer, R.
  • Paripovic, D.
  • Pavicevic, S.
  • Peco-Antic, A.
  • Podgoreanu, E.
  • Podracka, L.
  • Pokrajac, D.
  • Puretic, Z.
  • Puteo, F.
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