Characteristics and outcomes of children with primary oxalosis requiring renal replacement therapy.
OBJECTIVE
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.


METHODS
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.
ESPN/ERA-EDTA Registry, Department of Medical Informatics, Academic Medical Center, Amsterdam, The Netherlands.
Jérôme
J
Harambat
Karlijn J
KJ
van Stralen
Laura
L
Espinosa
Jaap W
JW
Groothoff
Sally-Anne
SA
Hulton
Rimante
R
Cerkauskiene
Franz
F
Schaefer
Enrico
E
Verrina
Kitty J
KJ
Jager
Pierre
P
Cochat
D000293:Adolescent; D000367:Age Factors; D002648:Child; D002675:Child, Preschool; D005060:Europe / Q000453:epidemiology; D005260:Female; D006801:Humans; D006960:Hyperoxaluria, Primary / Q000453:epidemiology / Q000401:mortality / Q000628:therapy*; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D053208:Kaplan-Meier Estimate; D007676:Kidney Failure, Chronic / Q000453:epidemiology / Q000401:mortality / Q000628:therapy*; D016030:Kidney Transplantation / Q000009:adverse effects / Q000401:mortality; D016031:Liver Transplantation; D008297:Male; D015995:Prevalence; D016016:Proportional Hazards Models; D012042:Registries; D006435:Renal Dialysis / Q000009:adverse effects / Q000401:mortality; D018570:Risk Assessment; D012307:Risk Factors; D013997:Time Factors; D016896:Treatment Outcome