Natural history of urine and plasma oxalate in children with primary hyperoxaluria type 1
Sas DJ, Mara K, Mehta RA, Seide BM, Banks CJ, Danese DS, et al.
Published in Pediatr Nephrol, Vol. 39 (1), pp. 141-148
Methodology
Retrospective registry study of 83 genetically confirmed PH1 patients from the Rare Kidney Stone Consortium PH Registry (2003-2018) with repeated urine or plasma oxalate before age 18, compared by G170R status before and after pyridoxine.
Key Findings
Children without G170R had the highest urine and plasma oxalate before and after B6. Homozygous G170R children had the lowest 24-hour urine oxalate and oxalate-to-creatinine ratio after B6. eGFR over time did not differ between groups.
Conclusions
Children under 5 with PH1 have relatively high urine oxalate; homozygous G170R may mean milder disease.
Limitations
Retrospective registry, no untreated comparison, genotype and B6 effects are intertwined.
Supplements Studied
Patients with homozygous G170R had the lowest 24-h Uox and Uox:cr after B6
Related Health Concerns
Study Details
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