Clinical Chemistry
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Clinical Chemistry 44: 463-471, 1998;
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(Clinical Chemistry. 1998;44:463-471.)
© 1998 American Association for Clinical Chemistry, Inc.

Molecular Pathology and Genetics

Simultaneous analysis of plasma free fatty acids and their 3-hydroxy analogs in fatty acid ß-oxidation disorders

Catarina G. Costa1,2,3, Lambertus Dorland3, Ulbe Holwerda2, Isabel Tavares de Almeida1, Bwee-Tien Poll-The3, Cornelis Jakobs2, and Marinus Duran3,a

1 Centro de Metabolismos e Genética, University of Lisboa, Lisbon 1699, Portugal.

2 Free University Hospital, Department of Clinical Chemistry (Metabolic Unit), Amsterdam, The Netherlands.

3 Wilhelmina Kinderziekenhuis, University of Utrecht, Utrecht, The Netherlands.
a Address correspondence to this author at: University Children's Hospital, "Het Wilhelmina Kinderziekenhuis", Nieuwe Gracht 137, 3512 LK Utrecht, The Netherlands. Fax 31-30-232 0793; e-mail m.duran{at}wkz.ruu.nl.

We present a new derivatization procedure for the simultaneous gas chromatographic–mass spectrometric analysis of free fatty acids and 3-hydroxyfatty acids in plasma. Derivatization of target compounds involved trifluoroacetylation of hydroxyl groups and tert-butyldimethylsilylation of the carboxyl groups. This new derivatization procedure had the advantage of allowing the complete baseline separation of free fatty acids and 3-hydroxyfatty acids while the superior gas chromatographic and mass spectrometric properties of tert-butyldimethylsilyl derivatives remained unchanged, permitting a sensitive analysis of the target compounds. Thirty-nine plasma samples from control subjects and patients with known defects of mitochondrial fatty acid ß-oxidation were analyzed. A characteristic increase of long-chain 3-hydroxyfatty acids was observed for all of the long-chain 3-hydroxyacyl-CoA dehydrogenase-deficient and mitochondrial trifunctional protein-deficient plasma samples. For medium-chain acyl-CoA dehydrogenase deficiency and very-long-chain acyl-CoA dehydrogenase deficiency, decenoic and tetradecenoic acids, respectively, were the main abnormal fatty acids, whereas the multiple acyl-CoA dehydrogenase-deficient patients showed variable increases of these unusual intermediates. The results showed that this selective and sensitive method is a powerful tool in the diagnosis and monitoring of mitochondrial fatty acid ß-oxidation disorders.




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