TY - JOUR
T1 - Characteristics and Outcomes of Children with Primary Oxalosis Requiring Renal Replacement Therapy
AU - Harambat, Jérôme
AU - van Stralen, Karlijn J.
AU - Espinosa, Laura
AU - Groothoff, Jaap W.
AU - Hulton, Sally-Anne
AU - Cerkauskiene, Rimante
AU - Schaefer, Franz
AU - Verrina, Enrico
AU - Jager, Kitty J.
AU - Cochat, Pierre
AU - AUTHOR GROUP
AU - Shitza, 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 - Lombaerts, R.
AU - Pokrajac, D.
AU - Roussinov, D.
AU - Puretić, Z.
AU - Batinić, D.
AU - Seeman, T.
AU - Vondrak, K.
AU - Heaf, J.
AU - Toots, U.
AU - Finne, P.
AU - Grönhagen-Riska, C.
AU - Holmberg, C.
AU - Couchoud, C.
AU - Niaudet, P.
AU - le Mignot, L.
AU - Sahpazova, E.
AU - Gersdorf, G.
AU - Scholz, C.
AU - Ioannidis, G.
AU - Reusz, G.
AU - Túri, S.
AU - Szabó, L.
AU - Szabó, T.
AU - Gianoglio, B.
AU - Leozappa, G.
AU - Mencarelli, F.
AU - Minale, B.
AU - Puteo, F.
AU - Jankauskiene, A.
AU - Schoenmaker, N.
AU - Kramer, A.
AU - van de Luijtgaarden, M. W.
PY - 2012
Y1 - 2012
N2 - 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. Clin J Am Soc Nephrol 7: 458-465, 2012. doi: 10.2215/CJN.07430711
AB - 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. Clin J Am Soc Nephrol 7: 458-465, 2012. doi: 10.2215/CJN.07430711
U2 - https://doi.org/10.2215/CJN.07430711
DO - https://doi.org/10.2215/CJN.07430711
M3 - Article
C2 - 22223608
SN - 1046-6673
VL - 7
SP - 458
EP - 465
JO - Clinical journal of the American Society of Nephrology
JF - Clinical journal of the American Society of Nephrology
IS - 3
ER -