Objective Hyperoxaluria leads to calcium oxalate crystal formation and subsequent urolithiasis. This study aims to analyse the effect of treatment compliance in hyperoxaluria, firstly by analysis of patients with non-primary hyperoxaluria and secondly via systematic review in patients with any hyperoxaluria. Methods In a retrospective cohort study, adults with non-primary hyperoxaluria managed with dietary counselling in 2013 were enrolled. Twenty-four-hour (24 h) urine collections initially and at 6 months were obtained. Compliance was assessed by self-reported dietary compliance and 24 h urinary volume >2 L. Patients were followed for 24 months. Primary outcomes were urinary oxalate and calcium 24 h load at 6 months, and urolithiasis-related procedural rates at 24 months. A Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA)-compatible systematic review of compliance among hyperoxaluric patients was performed. Results In the cohort study, of 19 eligible patients (4 female) with median age 52 years, 10 (53%) were considered compliant. Compared with the non-compliant group, these patients had significantly increased subsequent 24 h urinary volume (2250 mL vs. 1600 mL; p = 0.008) and lower procedural rates (10% vs. 56%; p = 0.033). Subsequent 24 h urinary oxalate load was non-significantly lower in compliant patients. Systematic review regarding compliance in hyperoxaluric patients revealed five studies. Only one utilised dietary counselling or analysed compliant vs. non-compliant patients, finding no difference. None examined the effect of compliance on procedural rates. Conclusion Hyperoxaluria is an important cause of recurrent urolithiasis. Increasing fluid intake and reducing dietary oxalate reduce the risk of operative intervention and remain fundamental to the treatment of hyperoxaluria.
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