Transplantation for Primary Hyperoxaluria Type 1: Designing New Strategies in the Era of Promising Therapeutic Perspectives

Devresse, Arnaud; Cochat, Pierre; Godefroid, Nathalie; Kanaan, Nada

doi:10.1016/j.ekir.2020.09.022

Cited by 45 publications

(44 citation statements)

References 50 publications

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“…These new medications will likely obviate the need for a liver transplant, but at first this will not be available for everyone. In addition, a kidney transplant will remain inevitable for patients who have already proceeded to CKD stage 5 [ 73 ]. This systematic review provides an overview of transplantation approaches in order to contribute to evidence-based decision-making.…”

Section: Discussionmentioning

confidence: 99%

Transplantation outcomes in patients with primary hyperoxaluria: a systematic review

et al. 2021

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Background Primary hyperoxaluria type 1 (PH1) is characterized by hepatic overproduction of oxalate and often results in kidney failure. Liver-kidney transplantation is recommended, either combined (CLKT) or sequentially performed (SLKT). The merits of SLKT and the place of an isolated kidney transplant (KT) in selected patients are unsettled. We systematically reviewed the literature focusing on patient and graft survival rates in relation to the chosen transplant strategy. Methods We searched MEDLINE and Embase using a broad search string, consisting of the terms ‘transplantation’ and ‘hyperoxaluria’. Studies reporting on at least four transplanted patients were selected for quality assessment and data extraction. Results We found 51 observational studies from 1975 to 2020, covering 756 CLKT, 405 KT and 89 SLKT, and 51 pre-emptive liver transplantations (PLT). Meta-analysis was impossible due to reported survival probabilities with varying follow-up. Two individual high-quality studies showed an evident kidney graft survival advantage for CLKT versus KT (87% vs. 14% at 15 years, p<0.05) with adjusted HR for graft failure of 0.14 (95% confidence interval: 0.05–0.41), while patient survival was similar. Three other high-quality studies reported 5-year kidney graft survival rates of 48–89% for CLKT and 14–45% for KT. PLT and SLKT yielded 1-year patient and graft survival rates up to 100% in small cohorts. Conclusions Our study suggests that CLKT leads to superior kidney graft survival compared to KT. However, evidence for merits of SLKT or for KT in pyridoxine-responsive patients was scarce, which warrants further studies, ideally using data from a large international registry.

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Section: Discussionmentioning

confidence: 99%

Transplantation outcomes in patients with primary hyperoxaluria: a systematic review

et al. 2021

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“…In individuals with stage 4 and 5 CKD, urinary oxalate excretion decreases and plasma oxalate starts to rise (20). Plasma oxalate levels are used to monitor primary hyperoxaluria patients with CKD and on dialysis, prior to transplantation (21)(22).…”

Section: Oxalate Metabolism and Measurementmentioning

confidence: 99%

“…When estimated glomerular filtration rate (eGFR) drops to ≤ 30-45 ml/min per 1.73m 2 , plasma oxalate increases, and oxalate may deposit in bone, kidneys, skin, retina, the cardiovascular and the central nervous systems. This dramatic condition is referred to as systemic oxalosis (2,(22)(23). Primary hyperoxaluria type 1 is the most common and severe form, generally leading to kidney failure during the first three decades of life.…”

Section: Primary Hyperoxaluriamentioning

confidence: 99%

“…Conservative therapeutic options in primary hyperoxaluria include massive fluid intake (tube or gastrostomy feeding in infants), calcium oxalate crystallization inhibitors, as well as vitamin B6 (pyridoxine) in primary hyperoxaluria type 1 (23). To date, liver transplantation is the only established 'curative' therapy to correct the metabolic defect contributing to endogenous oxalate formation (2,(22)(23). Liver-kidney transplantation (simultaneously or sequentially) is the current standard of care in patients with primary hyperoxaluria type 1 and CKD.…”

Section: Primary Hyperoxaluriamentioning

confidence: 99%

“…Liver-kidney transplantation (simultaneously or sequentially) is the current standard of care in patients with primary hyperoxaluria type 1 and CKD. It should ideally J o u r n a l P r e -p r o o f be performed prior to the development of systemic oxalosis and related complications (22)(23)(24). Indeed, outcome after kidney transplantation is improved by a substantial residual kidney function and by the absence of major systemic oxalate load (23).…”

Section: Primary Hyperoxaluriamentioning

confidence: 99%

See 2 more Smart Citations

Pathophysiology and Management of Hyperoxaluria and Oxalate Nephropathy: A Review

Demoulin

Aydın

Gillion

et al. 2022

American Journal of Kidney Diseases

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Impact of low‐oxalate diet on hyperoxaluria among patients suffering from nephrolithiasis

Aziz,

Noreen,

Tufail

et al. 2024

Food Science & Nutrition

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Low‐oxalate diets are useful for treating hyperoxaluria in nephrolithiasis patients. This study was unique in examining how a low‐oxalate diet in addition to a standard diet affected hyperoxaluria and renal function tests in nephrolithiasis patients. The effects of a low‐oxalate diet were analyzed by different biochemical tests, that is, anthropometric measurements, blood oxalate test, renal function test, electrolyte profile test, and 24 h urine analysis. For this purpose, 112 patients were divided into 2 groups: Group T1 (Conventional diet) and Group T2 (Low‐Oxalate diet) for 8 weeks. Each group was tested at the initiation and end of the study. Using SPSS, the obtained data from each parameter were statistically analyzed. The results showed that a low‐oxalate diet had a positive effect on patients suffering from nephrolithiasis. Furthermore, after treatment, anthropometric measurement weight (kg) among the control group (T1) was 100.45 ± 5.65 and the treatment group (T2) was 79.71 ± 9.48 kg. The effect of low‐oxalate diet on renal function test: creatinine (g/d) among T1 was 2.08 ± 0.86 and T2 was 1.17 ± 0.13, uric acid(mg/d) among T1 was 437.04 ± 24.20 and T2 was 364.61 ± 35.99, urinary oxalate (mg/d) among T1 was 76.84 ± 10.33 and T2 was 39.24 ± 1.51, respectively. Sodium (mEq/d) among T1 was 156.72 ± 6.37 and T2 was 159.84 ± 6.31, potassium (mEq/d) among T1 was 69.91 ± 15.37 and T2 was 89.21 ± 6.31, phosphorus (g/d) among T1 was 0.96 ± 0.07 and T2 was 0.34 ± 0.27, respectively. This study demonstrated that nephrolithiasis patients with hyperoxaluria benefit from low‐oxalate diets. Hyperoxaluria patients should eat a low‐oxalate diet to use oxalate without affecting metabolism and eliminate it from the kidney without stones.

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Transplantation for Primary Hyperoxaluria Type 1: Designing New Strategies in the Era of Promising Therapeutic Perspectives

Cited by 45 publications

References 50 publications

Transplantation outcomes in patients with primary hyperoxaluria: a systematic review

Transplantation outcomes in patients with primary hyperoxaluria: a systematic review

Pathophysiology and Management of Hyperoxaluria and Oxalate Nephropathy: A Review

Impact of low‐oxalate diet on hyperoxaluria among patients suffering from nephrolithiasis

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