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Hyperoxaluria

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 Hyperoxaluria Consortium (OxalEurope/ERKNet 2023), the Canadian Urological Association (CUA 2022), the European Association of Urology (EAU 2021), 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 Endocrinology (ACE/AACE 2016), the American Urological Association (AUA 2014), and the American Association for the Study of Liver Diseases (AASLD 2014).
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Guidelines

1.Screening and diagnosis

Indications for testing: 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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2.Diagnostic investigations

Urine oxalate studies: 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

3.Medical management

RNA interference therapy: 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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4.Nonpharmacologic interventions

Dietary oxalate restriction
As per ERKNet 2023 guidelines:
Initiate conservative therapy promptly in all patients with suspected primary hyperoxaluria.
B
Offer a balanced diet in patients with primary hyperoxaluria, avoiding only foods containing extremely high levels of oxalate.
B

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  • Fluid intake

  • Pyridoxine supplementation

  • Potassium supplementation

5.Therapeutic procedures

Kidney stone removal: 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

6.Surgical interventions

Kidney transplantation: as per ERKNet 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

7.Specific circumstances

Patients with infantile oxalosis: 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

8.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

9.Follow-up and surveillance

Assessment of treatment response
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