Characteristics and Outcomes of Children With Primary Oxalosis Requiring Renal Replacement Therapy

Clin J Am Soc Nephrol. 2012 Mar;7(3):458-65. doi: 10.2215/CJN.07430711. Epub 2012 Jan 5.

Abstract

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.

Publication types

  • Multicenter Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Age Factors
  • Child
  • Child, Preschool
  • Europe / epidemiology
  • Female
  • Humans
  • Hyperoxaluria, Primary / epidemiology
  • Hyperoxaluria, Primary / mortality
  • Hyperoxaluria, Primary / therapy*
  • Incidence
  • Infant
  • Infant, Newborn
  • Kaplan-Meier Estimate
  • Kidney Failure, Chronic / epidemiology
  • Kidney Failure, Chronic / mortality
  • Kidney Failure, Chronic / therapy*
  • Kidney Transplantation* / adverse effects
  • Kidney Transplantation* / mortality
  • Liver Transplantation
  • Male
  • Prevalence
  • Proportional Hazards Models
  • Registries
  • Renal Dialysis* / adverse effects
  • Renal Dialysis* / mortality
  • Risk Assessment
  • Risk Factors
  • Time Factors
  • Treatment Outcome