title: Propofol infusion syndrome.
abstract: The clinical features of propofol infusion syndrome (PRIS) are acute refractory bradycardia leading to asystole, in the presence of one or more of the following: metabolic acidosis (base deficit > 10 mmol.l(-1)), rhabdomyolysis, hyperlipidaemia, and enlarged or fatty liver. There is an association between PRIS and propofol infusions at doses higher than 4 mg.kg(-1).h(-1) for greater than 48 h duration. Sixty-one patients with PRIS have been recorded in the literature, with deaths in 20 paediatric and 18 adult patients. Seven of these patients (four paediatric and three adult patients) developed PRIS during anaesthesia. It is proposed that the syndrome may be caused by either a direct mitochondrial respiratory chain inhibition or impaired mitochondrial fatty acid metabolism mediated by propofol. An early sign of cardiac instability associated with the syndrome is the development of right bundle branch block with convex-curved ('coved type') ST elevation in the right praecordial leads (V1 to V3) of the electrocardiogram. Predisposing factors include young age, severe critical illness of central nervous system or respiratory origin, exogenous catecholamine or glucocorticoid administration, inadequate carbohydrate intake and subclinical mitochondrial disease. Treatment options are limited. Haemodialysis or haemoperfusion with cardiorespiratory support has been the most successful treatment.
authors.affiliation: Department of Anaesthetics, University of Sydney, Royal Prince Alfred Hospital, Camperdown, NSW 2050, Australia. pkam@usyd.edu.au
authors.forename: P C A
authors.initials: PC
authors.lastname: Kam
authors.forename: D
authors.initials: D
authors.lastname: Cardone
mesh_terms: D000138:Acidosis / Q000139:chemically induced; D000293:Adolescent; D000328:Adult; D018686:Anesthetics, Intravenous / Q000009:adverse effects; D015415:Biomarkers / Q000097:blood; D001919:Bradycardia / Q000139:chemically induced* / Q000503:physiopathology / Q000628:therapy; D002648:Child; D002675:Child, Preschool; D003645:Death, Sudden / Q000209:etiology; D005260:Female; D006801:Humans; D006993:Hypnotics and Sedatives / Q000009:adverse effects*; D007223:Infant; D008297:Male; D028361:Mitochondrial Diseases / Q000139:chemically induced; D015742:Propofol / Q000009:adverse effects*; D012307:Risk Factors; D013577:Syndrome
chemical_list: D018686:Anesthetics, Intravenous; D015415:Biomarkers; D006993:Hypnotics and Sedatives; D015742:Propofol