Valproic acid (VPA) is a widely used anticonvulsant drug which affects mitochondrial metabolism including the catabolism of fatty acids and branched-chain amino acids. 3-hydroxyisovaleric acid (3OH-IVA), one secondary metabolite of the leucine pathway, were significantly elevated in human urine after VPA treatment [Luis PB et al. 2012]
onset, adolescent
onset, childhood
onset, infancy
laboratory finding
3-Hydroxyisovaleric acid50.00500.00 mmol/mol Creaurine
Sodium120.00140.00 mmol/lserum
2-Methyl-3-hydroxybutyric acid mmol/mol Creaurine
Glycine300.003000.00 mmol/mol Creaurine
3-Hydroxyisovalerylcarnitine (C5-OH) +€mol/lplasma
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