Fine-tuning circulating oxalate levels to improve transplant strategies in primary hyperoxaluria: what is the ideal threshold in pediatrics?

Abstract

Background: Interfering RNA therapies (RNAi) have changed the management of patients with hyperoxaluria type 1 (PH1); data in dialysis remain scarce.

Results: A PH1 teenager undergoing intensive hemodiafiltration received lumasiran. POx levels almost halved during the loading phase (98 to 52 µmol/L), but rebound occurred when doses were quarterly-spaced, with POx at 94 µmol/L at 5 months. Lumasiran injections were therefore performed monthly, allowing adequate POx control (52 µmol/L) and isolated kidney transplantation. We also evaluated POx in 26 non-PH1 children with current dialysis techniques at a median(range) age of 10.9 (2.6-17.0) years, time on dialysis 14 (0-52) months, and POx 35 (8-125) µmol/L; residual diuresis was associated with lower POx. Circulating glycolate levels were normal in non-PH1 patients.

Conclusion: Intensification of lumasiran therapy is possible in dialysis and improves POx levels before kidney transplantation; POx levels in non-PH1 pediatrics patients in dialysis are provided to improve decision making in transplantation.