Propofol infusion syndrome is characterized by severe lactic acidosis, rhabdomyolysis and lipidemia which can lead to cardiovascular collapse.
The suggested dose and duration to avoid propofol infusion syndrome in healthy patients is > 4 mg/kg/h over 48 hours.
The syndrome may occur with smaller doses and duration in children with Mitochondrial diseases.The syndrome may occur with smaller doses and duration in children with underlying disorders (200 mcg/k/min over 150 min in a 7 y.o.)
Mitochondrial diseases affect ATP production and present as clinical disorders of skeletal muscle, brain and heart.
Propofol has several negative effects on mitochondrial ATP production which include:
1) inhibition of enzyme activity of electron transport complex
2) inhibition of carnitine palmitoyl transferase
3) inhibition of
These depressant mitochondrial effects may account for proposal infusion syndrome occurring in these susceptible
patients.Mitochondrial disorders have an incidence of one in 4000.
Also there are many case reports of anaphylaxis due to propofol in patients allergic to soya bean oil,or peanuts.
Faraq E, et al. Metabolic acidosis due to propofol infusion. Anesthesiology 2005;102:697-8.
Kill C, et al. Lacticacidosis after short-term infusion of propofol for anaesthesia in a child with
osteogenesis imperfecta. Paed Anaesth 2003;13:823-6.
Driesen J, et al. Anesthesia-related morbidity and mortality after surgery for muscle biopsy in children
with mitochondrial defects. Ped Anesth 2007;17:16-21.β-oxidation.
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