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Hyperoxaluria
Guidelines
Key sources
The following summarized guidelines for the evaluation and management of hyperoxaluria are prepared by our editorial team based on guidelines from the European Association of Urology (EAU 2025), the European Hyperoxaluria Consortium (OxalEurope/ERN ERKNet 2023), the Canadian Urological Association (CUA 2022), the American Society of Anesthesiologists (ASA/ACE/OS/AACE/ASMBS/OMA 2020), the Kidney Disease: Improving Global Outcomes Foundation (KDIGO 2020), the American College ...
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Screening and diagnosis
Indications for testing: as per AUA 2014 guidelines, obtain metabolic testing including urinary oxalate assessment (with one or two 24-hour urine collections obtained on a random diet) in patients with recurrent nephrolithiasis, or in high-risk or interested patients with a first episode of nephrolithiasis.
B
Diagnostic investigations
Urine oxalate studies: as per ERN ERKNet/OxalEurope 2023 guidelines, assess urinary oxalate excretion, along with creatinine, by 24-hour urine collection.
B
consider obtaining spot urine collections instead of 24-hour urine collections, where clinically necessary, provided that oxalate assessment is expressed as the oxalate-to-creatinine ratio. B
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Plasma oxalate levels
Genetic testing
Medical management
Nonpharmacologic interventions
Dietary oxalate restriction: as per EAU 2025 guidelines, advise oxalate restriction for the prevention of kidney stone formation in patients with hyperoxaluria. Advise reducing dietary fat and oxalate in patients with enteric hyperoxaluria.
B
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Dietary oxalate restriction (ERKNet)
Fluid intake
Pyridoxine supplementation
Potassium supplementation
Therapeutic procedures
Kidney stone removal: as per ERN ERKNet/OxalEurope 2023 guidelines, consider performing percutaneous nephrolithotomy or ureteroscopy rather than external shockwave lithotripsy for stone removal in patients with primary hyperoxaluria.
C
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Dialysis
Surgical interventions
Kidney transplantation: as per KDIGO 2020 guidelines, consider performing combined or sequential liver-kidney transplantation in eligible patients with primary hyperoxaluria type 1.
C
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Kidney transplantation (ERKNet)
Liver transplantation
Liver transplantation (ERKNet)
Specific circumstances
Patients with infantile oxalosis: as per ERN ERKNet/OxalEurope 2023 guidelines, define infantile oxalosis as stage 5D CKD due to primary hyperoxaluria before the age of 1 year.
B
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Patients with enteric hyperoxaluria
Patients with hyperuricosuria
Preventative measures
Follow-up and surveillance
Assessment of treatment response
As per ERN ERKNet/OxalEurope 2023 guidelines:
Assess pyridoxine responsiveness in all patients with primary hyperoxaluria type 1 and titrate its dose based on urinary oxalate excretion.
B
Consider continuing RNA interference therapy and other specific new therapies based on annual re-evaluation of biochemical and clinical efficacy.
C
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Imaging surveillance