TY  - CHAP
M1  - Book, Section
TI  - Acyl-Coa Dehydrogenase Deficiency Syndrome
A1  - Bissonnette, Bruno
A1  - Luginbuehl, Igor
A1  - Marciniak, Bruno
A1  - Dalens, Bernard J.
PY  - 2006
T2  - Syndromes: Rapid Recognition and Perioperative Implications
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".  
SN  - 
PB  - The McGraw-Hill Companies
CY  - New York, NY
Y2  - 2024/04/19
UR  - accessanesthesiology.mhmedical.com/content.aspx?aid=58061624
ER  -