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Hyperoxaluria

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Updated 2024 EAU guidelines for the management of 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 2024), the European Hyperoxaluria Consortium (OxalEurope/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 of ...
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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
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Diagnostic investigations

Urine oxalate studies: as per 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

RNA interference therapy: as per ERKNet/OxalEurope 2023 guidelines, consider weighing the benefit of RNA interference therapy against its potential long-term risks in patients with primary hyperoxaluria type 1.
C
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Nonpharmacologic interventions

Dietary oxalate restriction: as per EAU 2024 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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  • Fluid intake

  • Pyridoxine supplementation

  • Potassium supplementation

Therapeutic procedures

Kidney stone removal: as per ERKNet/OxalEurope 2023 guidelines, consider performing percutaneous nephrolithotomy or ureteroscopy rather than external shock wave lithotripsy for stone removal in patients with primary hyperoxaluria.
C

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  • Dialysis

Surgical interventions

Kidney transplantation: as per ERKNet/OxalEurope 2023 guidelines, decide on performing either sequential or simultaneous liver and kidney transplantation based on the clinical situation and the preference of the local surgeon.
B
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  • Liver transplantation

Specific circumstances

Patients with infantile oxalosis: as per 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

Medications to avoid: as per CUA 2022 guidelines, do not use vitamin C supplementation of > 1,000 mg/day because of the associated risk of hyperoxaluria and nephrolithiasis.
D

Follow-up and surveillance

Assessment of treatment response
As per 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