Abnormal fatty acid metabolism in patients in hopantenate therapy during clinical episodes.
Calcium 4-(2,4-dihydroxy-3,3-dimethylbutyramido)butyrate hemihydrate (hopantenate), a cerebral metabolic enhancer used in Japan since 1978, is a homologue of pantothenic acid. Using mass spectrometry, we found urinary excretion of 4-hydroxydodecanedioic acid, 4-hydroxytetradecanedioic acid and a series of 2-hydroxydicarboxylic acids (C8-C14), in addition to a series of odd- and even-numbered dicarboxylic acids (C5-C12) and 3-hydroxydicarboxylic acids (C8-C14) in patients receiving hopantenate during episodes of Reye's-like syndrome. Our findings suggest that an acute intoxication associated with hopantenate occurs owing to pantothenic acid deficiency or the inhibition of CoA-requiring reactions during stress, i.e. infection, prolonged fasting, or malnutrition.
Matsumoto
M
M
Division of Human Genetics, Kanazawa Medical University, Ishikawa, Japan.
Kuhara
T
T
Inoue
Y
Y
Shinka
T
T
Matsumoto
I
I
D000143:Acids / Q000652:urine; D000368:Aged; D002675:Child, Preschool; D005227:Fatty Acids / Q000378:metabolism*; D005260:Female; D008401:Gas Chromatography-Mass Spectrometry; D006801:Humans; D008297:Male; D013058:Mass Spectrometry; D010205:Pantothenic Acid / Q000009:adverse effects / Q000031:analogs & derivatives* / Q000627:therapeutic use; D012202:Reye Syndrome / Q000652:urine; D014297:Trimethylsilyl Compounds; D005680:gamma-Aminobutyric Acid / Q000009:adverse effects / Q000031:analogs & derivatives* / Q000627:therapeutic use
D000143:Acids; D005227:Fatty Acids; D014297:Trimethylsilyl Compounds; C016030:pantogab; D010205:Pantothenic Acid; D005680:gamma-Aminobutyric Acid