Effect of valproic acid on the urinary metabolic profile of a patient with succinic semialdehyde dehydrogenase deficiency

J Chromatogr B Analyt Technol Biomed Life Sci. 2003 Jul 15;792(1):99-106. doi: 10.1016/s1570-0232(03)00276-9.


The metabolic changes in a patient with succinic semialdehyde dehydrogenase deficiency were investigated following valproate administration using urease pretreatment and gas chromatography-mass spectrometry. A stable isotope dilution technique was used for quantification of urinary 4-hydroxybutyrate. Urinary levels of 4-hydroxybutyrate were 4-fold higher after 1-month valproate therapy. 4,5-Dihydrohexanoate, 2-deoxytetronate and 3-deoxytetronate were also 1.7-2.7-fold higher. The urinary excretions of 4-hydroxybutyrate in valproate non-medicated controls were age dependence and decreased with age. Relationships between 4-hydroxybutyrate excretion and 4-hydroxyvalproate or 5-hydroxyvalproate excretion were observed in valproate medicated controls. It seems that 4-hydroxyvalproate and 5-hydroxyvalproate as well as valproate are involved with increased excretion of 4-hydroxybutyrate following valproate administrations.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Aldehyde Oxidoreductases / urine*
  • Anticonvulsants / therapeutic use*
  • Gas Chromatography-Mass Spectrometry / methods
  • Humans
  • Hydroxybutyrates / urine
  • Infant
  • Male
  • Metabolism, Inborn Errors / drug therapy
  • Metabolism, Inborn Errors / urine*
  • Reproducibility of Results
  • Succinate-Semialdehyde Dehydrogenase
  • Valproic Acid / therapeutic use*


  • Anticonvulsants
  • Hydroxybutyrates
  • 4-hydroxybutyric acid
  • Valproic Acid
  • Aldehyde Oxidoreductases
  • ALDH5A1 protein, human
  • Succinate-Semialdehyde Dehydrogenase