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

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Tác giả: Cécile Acquaviva-Bourdain, Justine Bacchetta, Rouba Bechara, Julie Bernardor, Aurélie De Mul, Sacha Flammier, Marisca Carlina Makosso Afiavi, Anne-Laure Sellier-Leclerc, Ariane Zaloszyc

Ngôn ngữ: eng

Ký hiệu phân loại: 577.26 Autecology

Thông tin xuất bản: France : Nephrologie & therapeutique , 2025

Mô tả vật lý:

Bộ sưu tập: NCBI

ID: 687913

 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.
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