PT - JOURNAL ARTICLE AU - C. Largillière AU - C. Vianey-Saban AU - M. Fontaine AU - C. Bertrand AU - N. Kacet AU - J. P. Farriaux TI - Mitochondrial very long chain acyl-CoA dehydrogenase deficiency--a new disorder of fatty acid oxidation. AID - 10.1136/fn.73.2.F103 DP - 1995 Sep 01 TA - Archives of Disease in Childhood - Fetal and Neonatal Edition PG - F103--F105 VI - 73 IP - 2 4099 - http://fn.bmj.com/content/73/2/F103.short 4100 - http://fn.bmj.com/content/73/2/F103.full SO - Arch Dis Child Fetal Neonatal Ed1995 Sep 01; 73 AB - Very long chain acyl-CoA dehydrogenase is a newly characterised enzyme in mitochondrial fatty acid oxidation. A girl who presented on the second day of life with a sudden and severe illness due to deficiency of this enzyme is reported. There is evidence that some children (and perhaps all) originally diagnosed with a deficiency of long-chain acyl-CoA dehydrogenase, in fact, have a defect involving very long chain acyl-CoA dehydrogenase.