RT Book, Section
A1 Bissonnette, Bruno
A1 Luginbuehl, Igor
A1 Marciniak, Bruno
A1 Dalens, Bernard J.
SR Print(0)
ID 58061624
T1 Acyl-Coa Dehydrogenase Deficiency Syndrome
T2 Syndromes: Rapid Recognition and Perioperative Implications
YR 2006
FD 2006
PB The McGraw-Hill Companies
PP New York, NY
SN 9780071354554
LK accessanesthesiology.mhmedical.com/content.aspx?aid=58061624
RD 2024/04/18
AB Acylated  coenzyme  A  (acyl-CoA)  dehydrogenases  are  a  family  of  mitochondrial  enzymes  that  catalyze  the  first  dehydrogenation  step  in  the  beta  oxidation  of  fatty  acyl-CoA  derivatives.  Fatty  acids  provide  important  respiratory  fuel  for  many  tissues,  including  heart,  brain,  skeletal  muscle,  pancreas,  placenta,  brown  adipose  tissue,  kidney,  and  liver.  The  disorder  is  inherited  as  an  autosomal  recessive  trait.  Metabolic  acidosis,  severe  hypoglycemia  without  ketosis,  and  coma  are  frequent  features  of  all  types  of  acyl-CoA  dehydrogenase  deficiency  syndrome.  It  also  occurs  during  adulthood.  Large  amounts  of  glutaric  acid  in  the  blood  and  urine  are  caused  by  deficiency  of  the  enzyme  “multiple  acyl-CoA  dehydrogenase".