Impact of dietary ketosis on volatile anesthesia toxicity in a model of Leigh syndrome

• DOI: 10.1111/pan.14855
• Publication Date: 2024-02-15T14:49:30Z
• Authors: Kira A. Spencer; Miranda N. Howe; Michael T. Mulhol...; Vivian Truong; Ryan W. Liao; Yihan Chen; Monyreak Setha; John C. Snell; Allison Hanaford; Katerina James; Philip G. Morgan; Margaret M. Sedensky; Simon C. Johnson
• Keywords: Ketogenic Diet; Ketosis; Mitochondrial disease

Abstract Background Genetic mitochondrial diseases impact over 1 in 4000 individuals, most often presenting infancy or early childhood. Seizures are major clinical sequelae some including Leigh syndrome, the common pediatric presentation of disease. Dietary ketosis has been used to manage seizures disease patients. Mitochondrial patients require surgical interventions, leading anesthetic exposures. Anesthetics have shown be toxic setting disease, but a ketogenic diet on toxicities this not studied. Aims Our aim study was determine whether dietary impacts volatile genetic Methods The exposure studied by exposing young Ndufs4 (−/−) mice fed control isoflurane anesthesia. Blood metabolites were measured before and at end exposures, survival weight monitored. Results Compared regular diet, exacerbated hyperlactatemia resulting from (control vs. anesthesia mean difference 1.96 mM, Tukey's multiple comparison adjusted p = .0271) associated with significant increase mortality during immediately after exposures (27% 87.5% groups, respectively, period, Fisher's exact test .0121). data indicate that interact negatively Conclusions findings suggest extra caution should taken management ketosis.