Abstract: SA-PO0164
Effect of Oral Citrate Therapy on Urinary Biochemistry and Stone Outcomes in Patients with Primary Hyperoxaluria Type 3
Session Information
- Tubular, Metabolic, and Systemic Noncystic Genetic Kidney Diseases
October 24, 2026 | Location: Exhibit Hall A, Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Genetic Diseases of the Kidneys
- 1202 Genetic Diseases of the Kidneys: Non-Cystic (Complex and Non-Cystic Monogenic)
Authors
- Alshareef, Abdulmueti, Mayo Clinic Minnesota, Rochester, Minnesota, United States
- Arnous, Muhammad G., Mayo Clinic Minnesota, Rochester, Minnesota, United States
- Zaher, Ahmed Abdelnaby Aly Mahmoud, Mayo Clinic Minnesota, Rochester, Minnesota, United States
- Vaughan, Lisa E., Mayo Clinic Minnesota, Rochester, Minnesota, United States
- Sas, David J., Mayo Clinic Minnesota, Rochester, Minnesota, United States
- Milliner, Dawn S., Mayo Clinic Minnesota, Rochester, Minnesota, United States
- Lieske, John C., Mayo Clinic Minnesota, Rochester, Minnesota, United States
Group or Team Name
- Rare Kidney Stone Consortium
Background
Primary hyperoxaluria type 3 (PH3) is a rare genetic disorder characterized by recurrent calcium oxalate nephrolithiasis. Although oral citrate is commonly used to reduce lithogenic risk, its effect on biochemical and clinical outcomes in PH3 remains poorly defined.
Methods
We conducted a retrospective cohort study of genetically confirmed PH3 patients enrolled in the Rare Kidney Stone Consortium registry. Patients were categorized based on citrate exposure. Longitudinal associations between citrate use and urinary parameters were evaluated using generalized estimating equations adjusted for age and time since diagnosis. Among citrate users, within-patient comparisons of stone event rates before and during citrate therapy were assessed using the Wilcoxon signed-rank test.
Results
A total of 67 patients were included (39 citrate users, 28 non-users). In adjusted models citrate use was associated with significantly higher urinary citrate (119.5 mg/1.73 m2/24h; 95% CI 24.9–214.1; P=0.01) and increased urine pH (+0.31; 95% CI 0.16–0.46; P<0.001). However, calcium oxalate supersaturation did not significantly differ between groups. Among citrate users, within-patient comparisons demonstrated a significant reduction in stone event rates during citrate exposure compared to the pre-treatment period (median 0.22 vs 0.10 stone events/year; P=0.04).
Conclusion
Oral citrate therapy in this PH3 cohort was associated with favorable changes in urinary biochemistry and a reduction in stone activity in within-patient analyses. These findings support citrate as a key supportive therapy for PH3 patients, although prospective studies are needed to confirm its impact on clinical outcomes.
PH3 stone events over time
Funding
- NIDDK Support – Novo Nordisk