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Statements

Subject Item
n2:RIV%2F00064203%3A_____%2F12%3A8589%21RIV13-MZ0-00064203
rdf:type
n10:Vysledek skos:Concept
rdfs:seeAlso
http://dx.doi.org/10.2215/CJN.07430711
dcterms: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.
dcterms: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
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
skos:notation
RIV/00064203:_____/12:8589!RIV13-MZ0-00064203
n10:predkladatel
n18:ico%3A00064203
n3:aktivita
n16:I
n3:aktivity
I
n3:cisloPeriodika
3
n3:dodaniDat
n12:2013
n3:domaciTvurceVysledku
n9:5066093 n9:7517890
n3:druhVysledku
n8:J
n3:duvernostUdaju
n11:S
n3:entitaPredkladatele
n13:predkladatel
n3:idSjednocenehoVysledku
126780
n3:idVysledku
RIV/00064203:_____/12:8589
n3:jazykVysledku
n15:eng
n3:klicovaSlova
primary hyperoxaluria type-1; liver-kidney transplantation; allograft survival; united-states; registry; growth; experience; mutation; infants; disease
n3:klicoveSlovo
n6:allograft%20survival n6:infants n6:disease n6:experience n6:liver-kidney%20transplantation n6:primary%20hyperoxaluria%20type-1 n6:growth n6:registry n6:mutation n6:united-states
n3:kodStatuVydavatele
US - Spojené státy americké
n3:kontrolniKodProRIV
[C536DCC7B398]
n3:nazevZdroje
Clinical Journal of the American Society of Nephrology
n3:obor
n17:FE
n3:pocetDomacichTvurcuVysledku
2
n3:pocetTvurcuVysledku
89
n3:rokUplatneniVysledku
n12:2012
n3:svazekPeriodika
7
n3:tvurceVysledku
Podgoreanu, E. Pavicevic, S. Szabó, L. Puretic, Z. Leozappa, G. Hoeck, K. Podracka, L. Baiko, S. Espinosa, L. Sukalo, A. Seeman, Tomáš Reusz, G. Heaf, J. Zagozdzon, I. Túri, S. Grottes, JM Kolvek, G. Spasojevic-Dimitrijeva, B. Zurowska, A. Vondrák, Karel Oberbauer, R. Bikbov, BT Mircescu, G. Mencarelli, F. Verrina, E. Novljan, G. Afonso, C. Couchoud, C. Leivestad, T. Brackman, D. Garneata, L. Szabó, T. Grönhagen-Riska, C. Puteo, F. Scholz, C. Pokrajac, D. Batinic, D. Collart, F. Roussinov, D. Mignot, L. Molchanova, E. A. Groothoff, JW Sahpazova, E. Kramar, R. Hulton, S. A. Jäger, KJ Toots, U. Minale, B. Schaefer, F. Gersdorf, G. Jankauskiene, A. Paripovic, D. Battelino, N. Stralen, KJ Shitza, D. Finne, P. Gianoglio, B. … Cerkauskiene, R. Mota, C. Lombaerts, R. Almeida, M. Buturovic-Ponikvar, J. Gafencu, M. Cochat, P. Niaudet, P. Holmberg, C. Harambat, J. Kostic, M. Ioannidis, G. Tomilina, NA Peco-Antic, A.
n3:wos
000301222100014
s:issn
1555-9041
s:numberOfPages
8