title: Effect of a single dose of propofol and lack of dextrose administration in a child with mitochondrial disease: a case report.
abstract: Propofol infusion syndrome is a recognized complication of prolonged propofol use in the pediatric population, but little is reported on other metabolic effects of propofol, especially in children with mitochondrial disorders. We report on a child with metabolic encephalopathy, lactic acidosis, and stroke-like syndrome who received a single dose of propofol for procedural sedation. The patient's initial presentation was consistent with a mild exacerbation of her underlying disease. She received a single dose of propofol and non-dextrose-containing fluids during a magnetic resonance imaging (MRI) study to rule out stroke and progressed to develop severe acidosis, neurologic deterioration, and cardiorespiratory compromise. This is the first case report of severe metabolic disturbances after a single dose of propofol administered for procedural sedation in a patient with metabolic encephalopathy, lactic acidosis, and stroke-like syndrome and it questions the safety of propofol and absence of dextrose infusions during an acute illness in patients with mitochondrial disorders.
authors.affiliation: Department of Critical Care Medicine, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA Safar Center for Resuscitation Research, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA.
authors.forename: Haifa
authors.initials: H
authors.lastname: Mtaweh
authors.affiliation: Department of Critical Care Medicine, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA Department of Environmental and Occupational Health, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA Safar Center for Resuscitation Research, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA.
authors.forename: Hülya
authors.initials: H
authors.lastname: Bayır
authors.affiliation: Department of Critical Care Medicine, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA Safar Center for Resuscitation Research, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA.
authors.forename: Patrick M
authors.initials: PM
authors.lastname: Kochanek
authors.affiliation: Department of Critical Care Medicine, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA Department of Neurological Surgery, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA Safar Center for Resuscitation Research, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA bellmj4@upmc.edu.
authors.forename: Michael J
authors.initials: MJ
authors.lastname: Bell
mesh_terms: D000140:Acidosis, Lactic / Q000188:drug therapy / Q000209:etiology; D000293:Adolescent; D001794:Blood Pressure / Q000187:drug effects; D004569:Electroencephalography; D004827:Epilepsy / Q000188:drug therapy / Q000209:etiology; D005260:Female; D005947:Glucose / Q000008:administration & dosage; D006801:Humans; D006993:Hypnotics and Sedatives / Q000627:therapeutic use*; D008279:Magnetic Resonance Imaging; D028361:Mitochondrial Diseases / Q000150:complications / Q000188:drug therapy* / Q000235:genetics; D017354:Point Mutation / Q000235:genetics; D015742:Propofol / Q000627:therapeutic use*; D012356:RNA, Transfer, Leu / Q000235:genetics; D020521:Stroke / Q000209:etiology
chemical_list: D006993:Hypnotics and Sedatives; D012356:RNA, Transfer, Leu; D005947:Glucose; D015742:Propofol
keywords: MELAS; lactic acidosis; metabolic encephalopathy; mitochondrial disorders; propofol