Impaired fatty acid oxidation in propofol infusion syndrome

Area of Science:

• Critical Care Medicine
• Pediatric Intensive Care
• Biochemistry

Background:

• Propofol infusion syndrome (PRIS) is a rare but often fatal complication associated with prolonged infusions of propofol in critically ill pediatric patients.
• Early recognition and intervention are crucial for improving outcomes in PRIS.
• Understanding the underlying biochemical mechanisms of PRIS is essential for developing targeted therapies.

Observation:

• A pediatric patient receiving long-term propofol infusions presented with the full clinical spectrum of propofol infusion syndrome.
• The patient successfully responded to treatment with hemofiltration.
• Pre-hemofiltration biochemical analysis revealed significantly elevated plasma concentrations of malonylcarnitine and C5-acylcarnitine.

Findings:

• Elevated malonylcarnitine and C5-acylcarnitine levels normalized following hemofiltration and clinical recovery.
• These biochemical abnormalities suggest a disruption in fatty acid oxidation.
• The proposed mechanism involves impaired mitochondrial entry of long-chain acylcarnitine esters and complex II failure in the mitochondrial respiratory chain.

Implications:

• Hemofiltration may be an effective therapeutic option for managing propofol infusion syndrome.
• Monitoring specific acylcarnitine levels could aid in the diagnosis and management of PRIS.
• Further research into the mitochondrial dysfunction in PRIS is warranted to elucidate the precise pathogenic mechanisms.