Stiripentol and Lumasiran as a Rescue Therapy for Oxalate Nephropathy Recurrence After Kidney Transplantation in an Adult Patient With Primary Hyperoxaluria Type 1

Am J Kidney Dis. 2023 Jul;82(1):113-116. doi: 10.1053/j.ajkd.2022.12.005. Epub 2023 Jan 21.

Abstract

Primary hyperoxaluria type 1 is a rare cause of kidney failure. Stiripentol, an inhibitor of lactate dehydrogenase A, and lumasiran, a small interfering RNA targeting glycolate oxidase, have been proposed as therapeutic options, but clinical data are scarce, especially in adults and transplanted patients. We describe the case of a 51-year-old patient with a biopsy-proven recurrence of oxalate nephropathy after a kidney-only transplantation. He received stiripentol and lumasiran without adverse events. Fourteen months after transplantation, graft function, serum, and urinary oxalate levels have remained stable, and kidney biopsy showed a complete regression of oxalate crystals. Further studies are needed to assess whether this strategy is effective and could replace liver-kidney transplantation.

Keywords: primary hyperoxaluria, kidney transplantation, recurrence, lumasiran.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Humans
  • Hyperoxaluria* / etiology
  • Hyperoxaluria, Primary* / complications
  • Hyperoxaluria, Primary* / surgery
  • Kidney Transplantation* / adverse effects
  • Male
  • Middle Aged
  • Oxalates
  • RNA, Small Interfering
  • Renal Insufficiency* / etiology

Substances

  • stiripentol
  • lumasiran
  • RNA, Small Interfering
  • Oxalates

Supplementary concepts

  • Primary hyperoxaluria type 1