title: Impaired fatty acid oxidation in propofol infusion syndrome.
abstract: Propofol infusion syndrome is a rare but frequently fatal complication in critically ill children given long-term propofol infusions. We describe a child who developed all the clinical features of propofol infusion syndrome and was treated successfully with haemofiltration. Biochemical analysis before haemofiltration showed a large rise in plasma concentrations of malonylcarnitine (3.3 micromol/L) and C5-acylcarnitine (8.4 micromol/L), which returned to normal after recovery. Abnormalities are consistent with specific disruption of fatty-acid oxidation caused by impaired entry of long-chain acylcarnitine esters into the mitochondria and failure of the mitochondrial respiratory chain at complex 11.
authors.forename: A
authors.initials: A
authors.lastname: Wolf
authors.forename: P
authors.initials: P
authors.lastname: Weir
authors.forename: P
authors.initials: P
authors.lastname: Segar
authors.forename: J
authors.initials: J
authors.lastname: Stone
authors.forename: J
authors.initials: J
authors.lastname: Shield
mesh_terms: D000138:Acidosis / Q000139:chemically induced* / Q000378:metabolism / Q000628:therapy*; D002331:Carnitine / Q000097:blood; D003422:Critical Care; D004579:Electron Transport / Q000187:drug effects; D005227:Fatty Acids / Q000378:metabolism*; D006440:Hemofiltration; D006801:Humans; D006993:Hypnotics and Sedatives / Q000008:administration & dosage / Q000009:adverse effects*; D007223:Infant; D007262:Infusions, Intravenous; D008297:Male; D010084:Oxidation-Reduction; D015742:Propofol / Q000008:administration & dosage / Q000009:adverse effects*; D013577:Syndrome
chemical_list: D005227:Fatty Acids; D006993:Hypnotics and Sedatives; D002331:Carnitine; D015742:Propofol