Kidney Transplantation in Primary Oxalosis: Data From the EDTA Registry

Broyer, M.; Brunner, F; Brynger, H; Dykes, S. R.; Ehrich, J. H. H.; Faßbinder, W.; Geerlings, W.; Rizzoni, G; Selwood, N. H.; Tufveson, Gunnar; Wing, A. J.

doi:10.1093/ndt/5.5.332

Cited by 119 publications

(37 citation statements)

References 7 publications

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“…The ultimate management is centered on organ transplantation. Isolated kidney transplantation can reduce plasma oxalate levels and has been reported in the United States and in Europe (with fewer living donors) (11)(12)(13); however, disease recurrence often leads to poor graft survival. Liver transplantation completely corrects the enzyme defect.…”

Section: Introductionmentioning

confidence: 99%

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

Harambat

Stralen

Espinosa

et al. 2012

Clinical Journal of the American Society of Nephrology

134

105

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SummaryBackground 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. ConclusionsThe outcomes of PH children with ESRD are still poorer than in non-PH children but have substantially improved over time.

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Section: Introductionmentioning

confidence: 99%

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

Harambat

Stralen

Espinosa

et al. 2012

Clinical Journal of the American Society of Nephrology

134

105

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“…Similarly disappointing results with isolated renal transplantation caused by disease recurrence and the inefficiency of oxalate removal by standard modes of dialysis 9 were noted by other investigators. 10,11 Nonetheless, with advances in transplantation and increasing understanding of the pathophysiological course of PH over the past two decades, improved outcomes have been obtained in PH-I. 12,13 Enhanced awareness of the toxic effects and distribution of oxalate launched therapeutic tactics designed to minimize oxalate-related tissue damage.…”

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confidence: 99%

Combined liver-kidney and kidney-alone transplantation in primary hyperoxaluria

Monico

Milliner

2001

Liver Transplantation

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Combined liver-kidney and kidney-only transplantation outcomes in primary hyperoxaluria (PH) are described. Strategies for the selection of type and timing of transplantation and pretransplantation and posttransplantation management are reviewed. Records were reviewed for 16 patients with PH who received 9 liver-kidney and 10 kidney-only transplants. Plasma oxalate values declined from 61 ؎ 42 mol/L pretransplantation to 9 ؎ 6 mol/L 1 month after transplantation in liver-kidney transplant recipients and 92 ؎ 19 to 9 ؎ 5 mol/L in kidney-only transplant recipients. In most liver-kidney transplant recipients, hyperoxaluria persisted for 6 to 18 months after transplantation. Follow-up was 3.5 ؎ 4.1 years in liver-kidney and 4.5 ؎ 6.3 years in kidney-alone transplant recipients. Patient survival rates were 78% for liver-kidney and 89% for kidney-only transplant recipients. No hepatic allografts were lost. Three of 9 liverkidney and 6 of 10 kidney-alone transplants lost renal allograft function. In those with functioning kidneys, renal clearance was 45.1 ؎ 19.5 mL/min/1.73 m 2 in liverkidney transplant recipients and 49.5 ؎ 26.1 mL/min/ 1.73 m 2 in kidney-only transplant recipients at last follow-up. Kaplan-Meier 1-, 2-, 3-, and 5-year renal allograft survival rates for patients undergoing transplantation after 1984 were 78%, 78%, 52%, and 52% in liver-kidney transplant recipients and 86%, 71%, 54%, and 36% in kidney-only transplant recipients. Simultaneous grafting of liver and kidney after the development of renal insufficiency is recommended for the majority of patients with PH type I (PH-I). Kidney-alone transplantation is recommended for those with pyridoxine-responsive type I disease because pharmacological therapy allows favorable management of oxalate production in this situation. Kidney-alone transplantation also is recommended for PH type II (PH-II). This disease is less severe than PH-I, and it is currently unknown whether liver transplantation will correct the metabolic defect responsible for PH-II. (Liver Transpl 2001;7:954-963.)

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“…The experience with organ transplantation in PH I has been variable. Recurrence of oxalosis following kidney transplantation alone is anticipated since the underlying biochemical defect is not corrected (Broyer et al 1990). Combined LK transplantation has been advocated for patients with PH I by the European transplant registry Broyer et al 1990).…”

Section: Introductionmentioning

confidence: 99%

“…Recurrence of oxalosis following kidney transplantation alone is anticipated since the underlying biochemical defect is not corrected (Broyer et al 1990). Combined LK transplantation has been advocated for patients with PH I by the European transplant registry Broyer et al 1990). However, a report analyzing data from the United States Renal Data System has recommended that kidney transplantation alone could be a first option for patients with PH I (Saborio and Scheinman 1999).…”

Section: Introductionmentioning

confidence: 99%

Hyperoxaluria and Rapid Development of Renal Failure Following a Combined Liver and Kidney Transplantation: Emphasis on Sequential Transplantation

2011

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Primary hyperoxaluria type I (PH I) is a rare genetic disorder that leads to end stage renal disease (ESRD) at an early age due to excessive deposition of calcium oxalate in the kidney. Combined liver-kidney transplantation (LKTx) has been advocated as the treatment of choice for patients with PH I who have progressive renal disease. With combined LKTx the risk of early renal failure secondary to oxalate deposition is anticipated. Here we report a patient with PH I who developed ESRD and underwent a combined LKTx. He lost the kidney graft secondary to early recurrence of oxalosis. Repeat kidney transplantation 13 months after the initial procedure was successful. Elevated plasma oxalate levels persisted for a long time following LKTx and lead to further deposition of oxalate in the second kidney graft. Combined LKTx for patients with PH I requires meticulous preparation and very careful post operative management. Sequential liver transplantation followed by kidney transplantation is to be considered for PH I patients who have ESRD and very high oxalate load.

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Kidney Transplantation in Primary Oxalosis: Data From the EDTA Registry

Cited by 119 publications

References 7 publications

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

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

Combined liver-kidney and kidney-alone transplantation in primary hyperoxaluria

Hyperoxaluria and Rapid Development of Renal Failure Following a Combined Liver and Kidney Transplantation: Emphasis on Sequential Transplantation

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