PMID- 11750281
OWN - NLM
STAT- MEDLINE
DCOM- 20021104
LR  - 20190706
IS  - 0009-8981 (Print)
IS  - 0009-8981 (Linking)
VI  - 316
IP  - 1-2
DP  - 2002 Feb
TI  - 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.
PG  - 117-21
AB  - 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.
FAU - Kimura, Masahiko
AU  - Kimura M
AD  - Department of Pediatrics, Shimane Medical University, 89-1 Enya, Izumo, Shimane 
      693-8501, Japan. kimura@shimane-med.ac.jp
FAU - Yoon, Hye Ran
AU  - Yoon HR
FAU - Wasant, Pornswan
AU  - Wasant P
FAU - Takahashi, Yukitoshi
AU  - Takahashi Y
FAU - Yamaguchi, Seiji
AU  - Yamaguchi S
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - Netherlands
TA  - Clin Chim Acta
JT  - Clinica chimica acta; international journal of clinical chemistry
JID - 1302422
RN  - 0 (Fatty Acids)
RN  - EC 1.3.8.7 (Acyl-CoA Dehydrogenase)
RN  - EC 1.3.8.8 (Acyl-CoA Dehydrogenase, Long-Chain)
SB  - IM
MH  - Acyl-CoA Dehydrogenase
MH  - Acyl-CoA Dehydrogenase, Long-Chain/*deficiency
MH  - Blood Specimen Collection/methods
MH  - Child
MH  - Fatty Acids/*blood
MH  - Gas Chromatography-Mass Spectrometry/methods/standards
MH  - Humans
MH  - Mitochondrial Diseases/blood/*diagnosis/*enzymology
MH  - Oxidation-Reduction
MH  - Sensitivity and Specificity
EDAT- 2001/12/26 10:00
MHDA- 2002/11/26 04:00
CRDT- 2001/12/26 10:00
PHST- 2001/12/26 10:00 [pubmed]
PHST- 2002/11/26 04:00 [medline]
PHST- 2001/12/26 10:00 [entrez]
AID - S0009898101007410 [pii]
AID - 10.1016/s0009-8981(01)00741-0 [doi]
PST - ppublish
SO  - Clin Chim Acta. 2002 Feb;316(1-2):117-21. doi: 10.1016/s0009-8981(01)00741-0.