The Propofol Infusion Syndrome describes a potentially fatal condition occurring in children in the intensive care unit (ICU) receiving a propofol infusion for sedation. Because of concern for the syndrome multiple countries (US FDA, Canadians, others) have banned the use of propofol for use as a sedating agent in the ICU for children <= 18 years of age.
Features of the propofol infusion syndrome may include one or more of the following:
(1) bradycardia, asystole or cardiac conduction abnormalities
(2) heart failure or hemodynamic instability
(3) severe metabolic acidosis
(4) lipemia
(5) hepatomegaly
(6) rhabdomyolysis
The metabolic abnormality may be caused by impaired mitochondrial fatty acid oxidation (Wolf et al). Patients at risk for the syndrome are those with metabolic stress associated with starvation and/or serious infection. Wolf et al suggest that a carbohydrate infusion of 6-8 mg per kg per minute is sufficient to suppress fat metabolism in children and may prevent the syndrome. (ASIDE: Considering the thousands of children who received the drug and the relatively small number of fatal cases, could the affected children had an inherited metabolic defect that predisposed them to the syndrome?)
This reaction has not be described in children receiving propofol as a single bolus or short-term infusion.
Differential diagnosis:
(1) shock due to other conditions
(2) viral myocarditis
(3) fat overload syndrome
(4) pancreatitis
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