Impaired fatty acid oxidation in propofol infusion syndrome.
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.[1]References
- Impaired fatty acid oxidation in propofol infusion syndrome. Wolf, A., Weir, P., Segar, P., Stone, J., Shield, J. Lancet (2001) [Pubmed]
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