Publication: A sensitive and simplified method to analyze free fatty acids in children with mitochondrial beta oxidation disorders using gas chromatography/mass spectrometry and dried blood spots
2
Issued Date
2002-01-12
Resource Type
ISSN
00098981
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2-s2.0-0036135289
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Mahidol University
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SCOPUS
Bibliographic Citation
Clinica Chimica Acta. Vol.316, No.1-2 (2002), 117-121
Suggested Citation
Masahiko Kimura, Hye Ran Yoon, Pornswan Wasant, Yukitoshi Takahashi, Seiji Yamaguchi A sensitive and simplified method to analyze free fatty acids in children with mitochondrial beta oxidation disorders using gas chromatography/mass spectrometry and dried blood spots. Clinica Chimica Acta. Vol.316, No.1-2 (2002), 117-121. doi:10.1016/S0009-8981(01)00741-0 Retrieved from: https://repository.li.mahidol.ac.th/handle/123456789/20089
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Title
A sensitive and simplified method to analyze free fatty acids in children with mitochondrial beta oxidation disorders using gas chromatography/mass spectrometry and dried blood spots
Abstract
Background: A precise diagnosis of mitochondrial fatty acid beta-oxidation (FAO) disorders can be difficult as several enzymatic reactions are involved. Methods: Using 5 blood spots on filter paper, each 3 mm in diameter, octanoate, decanoate, cis-4-decenoic acid (C10:1) and cis-5-tetradecenoic acid (C14:1) were measured by one step transmethylation and gas chromatography-mass spectrometry (GC/MS). Results: In subjects with medium-chain acyl-CoA dehydrogenase (MCAD) deficiency C10:1 was increased. C14:1 was increased in very long-chain acyl-CoA dehydrogenase (VLCAD) deficiency, and both were increased in multiple acyl CoA dehydrogenase (MAD) deficiency. Conclusions: Free fatty acids (FFAs) can be measured with a small amount of blood sample if selective ion monitoring (SIM) in GC/MS analysis is used. A single microtube was sufficient throughout the procedure prior to injection onto GC/MS. © 2002 Elsevier Science B.V. All rights reserved.