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Description
| - 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)
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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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skos:prefLabel
| - 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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skos:notation
| - RIV/00064203:_____/12:8589!RIV13-MZ0-00064203
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http://linked.open...avai/predkladatel
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http://linked.open...avai/riv/aktivita
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http://linked.open...avai/riv/aktivity
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http://linked.open...iv/cisloPeriodika
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http://linked.open...vai/riv/dodaniDat
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http://linked.open...aciTvurceVysledku
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http://linked.open.../riv/druhVysledku
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http://linked.open...iv/duvernostUdaju
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http://linked.open...titaPredkladatele
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http://linked.open...dnocenehoVysledku
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http://linked.open...ai/riv/idVysledku
| - RIV/00064203:_____/12:8589
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http://linked.open...riv/jazykVysledku
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http://linked.open.../riv/klicovaSlova
| - primary hyperoxaluria type-1; liver-kidney transplantation; allograft survival; united-states; registry; growth; experience; mutation; infants; disease (en)
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http://linked.open.../riv/klicoveSlovo
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http://linked.open...odStatuVydavatele
| - US - Spojené státy americké
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http://linked.open...ontrolniKodProRIV
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http://linked.open...i/riv/nazevZdroje
| - Clinical Journal of the American Society of Nephrology
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http://linked.open...in/vavai/riv/obor
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http://linked.open...ichTvurcuVysledku
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http://linked.open...cetTvurcuVysledku
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http://linked.open...UplatneniVysledku
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http://linked.open...v/svazekPeriodika
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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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