Zespół popropofolowy u czteroletniej dziewczynki – opis przypadku

*Magdalena Mierzewska-Schmidt, Małgorzata Arłukiewicz-Piwowar, Marcin Rawicz, Bogumiła Wołoszczuk-Gębicka

Summary
Background.Propofol infusion syndrome (PRIS), characterized by cardiovascular collapse, metabolic acidosis, hepatomegaly, renal failure and rhabdomyolysis, is a rare and often fatal syndrome seen in critically ill patients undergoing long-term propofol sedation. Propofol may impair mitochondrial free fatty acid â-oxidation and mitochondrial respiratory chain function, resulting in an imbalance between energy demand and utilization, thus compromising cardiac and peripheral muscle cell function.
Case report. We describe a fatal case of PRIS that developed in a 4-yr-old girl, sedated with propofol over 51 hours for prolonged intubation due to epiglottitis. Metabolic acidosis was first noted after 27 hours of sedation and was misdiagnosed as the early signs of septicemia. After 48 hours of treatment the patient developed tachycardia, hypotension and hepatomegaly. The serum was found to be lipemic. The PRIS was recognized and propofol was discontinued. The severe metabolic acidosis was associated with ketonuria, prolonged INR 2.26, GOT 2694 U l^-1, GPT 746 U l^-1 and severe hyperkalemia 7.15 mmol l-1. The latter was a probable result of rhabdomyolysis, although specific markers were not taken. The child died because of extreme metabolic acidosis and cardiovascular collapse that progressed to defibrillation-resistant ventricular fibrillation.
Discussion. This case shows an association between prolonged propofol use and metabolic acidosis, rhabdomyolysis, and death in children. Risk factors for propofol infusion syndrome include high dose, and administration of more than 24 hours duration. Creatine phosphokinase, lactic acid levels, electrolytes, and arterial blood gases should be monitored frequently. If this syndrome is suspected, propofol should be discontinued immediately and haemodialysis should be considered. Potential mechanisms of the syndrome and ways to avoid it are discussed.
Conclusion. Propofol should be used with caution for sedation in critically ill patients, as well as for long-term anaesthesia in otherwise healthy children. Doses exceeding 4 mg kg^-1 h^-1 should be avoided. Presently, propofol is not recommended for long-term sedation in children.

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