3-Hydroxyoctanoic aciduria: Identification of a new organic acid in the urine of a patient with non-ketotic hypoglycemia

Richard I. Kelley, D. Holmes Morton

Research output: Contribution to journalArticlepeer-review

Abstract

A four-month-old child with non-ketotic hypoglycemia and rapidly progressive cirrhosis excreted in her urine large amounts of two unidentified organic acids in addition to a spectrum of saturated, unsaturated, and 3-hydroxy dicarboxylic acids in her urine. Gas chromatography mass spectrometry of the trimethylsilyl derivative of one of the unknown compounds suggested the structure of 3-hydroxyoctanoic acid, which was confirmed by similar analysis of the authentic compound. The same organic acid was found in the child's plasma. The significance of 3-hydroxyoctanoic aciduria as a possible marker for a primary defect of 3-hydroxy fatty acid metabolism is discussed.

Original languageEnglish (US)
Pages (from-to)19-26
Number of pages8
JournalClinica Chimica Acta
Volume175
Issue number1
DOIs
StatePublished - Jun 30 1988

Keywords

  • 3-Hydroxyacyl-coenzyme A dehydrogenase
  • 3-Hydroxyoctanoic acid
  • Dicarboxylic acid
  • Fatty acid metabolism
  • Hypoglycemia
  • beta-Oxidation

ASJC Scopus subject areas

  • Biochemistry
  • Clinical Biochemistry
  • Biochemistry, medical

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