ArticleThe Journal of clinical investigation2019
Stiripentol protects against calcium oxalate nephrolithiasis and ethylene glycol poisoning.
Article in The Journal of clinical investigation, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 34 papers.
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Who cites it
34 citing papers in PubMed.
- Animal Models of Hyperoxaluria and Their Relevance for Fundamental Research and Clinical Practice.International journal of molecular sciences · 2026Review
- Clinical Approaches and Emerging Therapeutic Horizons in Primary Hyperoxaluria.Journal of clinical medicine · 2026Review
- A Targeted Release Capsule of Lanthanum Carbonate: a New Efficient Cheap Treatment for Primary Hyperoxalurias.Kidney international reports · 2025Article
- Human glyoxylate metabolism revisited: New insights pointing to multi-organ involvement with implications for siRNA-based therapies in primary hyperoxaluria.Journal of inherited metabolic disease · 2025Review
- Synthesis andInternational journal of molecular sciences · 2024Article
- Second transplantation after kidney graft loss in primary hyperoxaluria type 2: a pedigree study and mutation analysis.Renal failure · 2024Article
- Mutation Characteristics of Primary Hyperoxaluria in the Chinese Population and Current International Diagnosis and Treatment Status.Kidney diseases (Basel, Switzerland) · 2024Review
- Navigating the Evolving Landscape of Primary Hyperoxaluria: Traditional Management Defied by the Rise of Novel Molecular Drugs.Biomolecules · 2024Review
- An Update on Stiripentol Mechanisms of Action: A Narrative Review.Advances in therapy · 2024Review
- Meta-data analysis of kidney stone disease highlights ATP1A1 involvement in renal crystal formation.Redox biology · 2023Article
- Clinical practice recommendations for primary hyperoxaluria: an expert consensus statement from ERKNet and OxalEurope.Nature reviews. Nephrology · 2023Review
- Application of metabolomics in urolithiasis: the discovery and usage of succinate.Signal transduction and targeted therapy · 2023Article
- Review
- Primary hyperoxaluria type 1: novel therapies at a glance.Clinical kidney journal · 2022Review
- Treatment of primary hyperoxaluria type 1.Clinical kidney journal · 2022Review
- Treatment with stiripentol in a patient with primary hyperoxaluria type 1: lesson for the clinical nephrologist.Journal of nephrology · 2022Article
- The effect of lumasiran therapy for primary hyperoxaluria type 1 in small infants.Pediatric nephrology (Berlin, Germany) · 2022Article
- Perspectives in primary hyperoxaluria - historical, current and future clinical interventions.Nature reviews. Urology · 2022Review
- Oxalate nephropathy: a review.Clinical kidney journal · 2022Article
- Lumasiran in the Management of Patients with Primary Hyperoxaluria Type 1: From Bench to Bedside.International journal of nephrology and renovascular disease · 2022Review
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15 authors.
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No grant is acknowledged in the PubMed record.
Abstract
Increased urinary oxalate excretion (hyperoxaluria) promotes the formation of calcium oxalate crystals. Monogenic diseases due to hepatic enzymes deficiency result in chronic hyperoxaluria, promoting end-stage renal disease in children and young adults. Ethylene glycol poisoning also results in hyperoxaluria promoting acute renal failure and frequently death. Stiripentol is an antiepileptic drug used to treat children affected by Dravet syndrome, possibly by inhibiting neuronal lactate dehydrogenase 5 isoenzyme. As this isoenzyme is also the last step of hepatic oxalate production, we hypothesized that Stiripentol would potentially reduce hepatic oxalate production and urine oxalate excretion. In vitro, Stiripentol decreased in a dose-dependent manner the synthesis of oxalate by hepatocytes. In vivo, Stiripentol oral administration reduced significantly urine oxalate excretion in rats. Stiripentol protected kidneys against calcium oxalate crystal deposits in acute ethylene glycol intoxication and chronic calcium oxalate nephropathy models. In both models, Stiripentol improved significantly renal function. Patients affected by Dravet syndrome and treated with Stiripentol had a lower urine oxalate excretion than control patients. A young girl affected by severe type I hyperoxaluria received Stiripentol for several weeks: urine oxalate excretion decreased by two-thirds. Stiripentol is a promising potential therapy against genetic hyperoxaluria and ethylene glycol poisoning.
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