Mass spectrometric identification of 2-hydroxydodecanedioic acid and its homologues in urine from patients with hopantenate therapy during clinical episode.

@article{Matsumoto1990MassSI,
  title={Mass spectrometric identification of 2-hydroxydodecanedioic acid and its homologues in urine from patients with hopantenate therapy during clinical episode.},
  author={Masahiro Matsumoto and Tomiko Kuhara and Yoshito Inoue and Toshihiro Shinka and Isamu Matsumoto and M Kajita},
  journal={Biomedical \& environmental mass spectrometry},
  year={1990},
  volume={19 3},
  pages={
          171-5
        }
}
Urine from patients with calcium-4-(2,4-dihydroxy-3,3-dimethylbutyramido) butyrate hemihydrate (hopantenate) therapy during episodes of Reye's-like syndrome was found to contain a number of unusual dicarboxylic acids in high concentrations; odd- and even-numbered medium-chain dicarboxylic acids, alpha-hydroxydicarboxylic acids and beta-hydroxydicarboxylic acids. The abnormal excretion of dicarboxylic acids, alpha- and beta-hydroxydicarboxylic acids disappeared after discontinuance of… 
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TLDR
It is suggested that the 3-hydroxy dicarboxylic acids are formed from fatty acids by a combination of omega-oxidation and incomplete beta-Oxidation, both of which are found in ketoacidosis.
Mass spectrometric identification of 2-hydroxy-sebacic acid in the urines of patients with neonatal adrenoleukodystrophy and Zellweger syndrome.
TLDR
The urines of children with neonatal adrenoleukodystrophy and Zellweger syndrome contained an excess of unusual even- and odd-numbered dicarboxylic acids with a chain length of from 5 to 15 carbon atoms, as well as 2-hydroxy-compounds, which appears as an additional useful marker of these peroxisomal disorders.
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The urine of a young child with hypoglycemia and a Reye's like syndrome contained an excess of unusual aromatic products with a three carbon chain, phenylpropionylglycine and 3- and 4-hydroxyphenyl)propionic acids, as well as of organic acids usually found in fatty acid beta-oxidation defects.
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