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Characteristics and outcomes of children with primary oxalosis requiring renal replacement therapy
Jérôme Harambat1, Karlijn J van Stralen, Laura Espinosa
1ESPN/ERA-EDTA Registry, Department of Medical Informatics, Academic Medical Center, Amsterdam, The Netherlands.
Insights
Outcomes for children with primary hyperoxaluria (PH) and end-stage renal disease (ESRD) are poorer than for other children, but have improved significantly over time, especially with liver-kidney transplants.
Area of Science:
- Pediatric Nephrology
- Renal Replacement Therapy
- Organ Transplantation
Background:
- Primary hyperoxaluria (PH) is a rare genetic disorder leading to kidney stone formation and potential end-stage renal disease (ESRD).
- Outcomes for children with PH causing ESRD are historically poor, with limited data on management and prognosis.
- Understanding the long-term outcomes of PH in pediatric ESRD is crucial for improving patient care.
Purpose of the Study:
- To analyze the management and outcomes of children with PH requiring renal replacement therapy (RRT).
- To compare the survival and transplantation rates of PH patients with non-PH patients.
- To identify trends in outcomes for pediatric PH patients over time.
Main Methods:
- Retrospective analysis of pediatric patients (<19 years) initiating RRT between 1979-2009.
- Data sourced from a large European registry across 31 countries.
- Comparison of survival and graft survival rates between PH and non-PH patients, including kidney and liver-kidney transplant recipients.
Main Results:
- 100 PH patients were identified among 9247 incident RRT patients; PH patients were significantly younger at RRT initiation.
- PH patients had a three-fold increased risk of death compared to non-PH patients, though survival rates improved over time for both groups.
- Liver-kidney transplantation showed better graft survival (82% at 5 years) compared to kidney-only transplantation (14% at 5 years) in PH patients, though both were lower than non-PH patients.
Conclusions:
- Pediatric PH patients with ESRD still face poorer outcomes than non-PH patients.
- Significant improvements in survival and transplantation outcomes for PH patients have been observed over recent decades.
- Liver-kidney transplantation appears to offer better graft survival for PH patients compared to kidney-only transplantation.
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.
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