title: [Anesthesia in a patient with carnitine deficiency syndrome].
abstract: A patient with carnitine deficiency syndrome for tracheostomy and resection of tracheal granuloma was anesthetized with nitrous oxide and sevoflurane and vecuronium bromide. Potentiation of effect of vecuronium was minimal, and anesthetic course was uneventful. Carnitine is essential for transportation of long chain fatty acids into mitchondrial matrix. Its deficiency causes metabolic failure in skeletal muscle and accumulation of acyl-CoA in central nervous system. Carnitine deficiency syndrome is associated with a number of metabolic disorders of fatty acid. Signs and symptoms of the syndrome include skeletal muscle weakness, cardiomyopathy, liver disfunction, central nervous system disorders, hypoketotic hypoglycemia and so forth. Since fasting, infection, or shivering have been shown to deteriorate carnitine deficiency syndrome, there is a risk of triggering the acute episode perioperatively. Moreover, there is a possibility of prolonged effect of muscle relaxants and delay in emergence from anesthesia due to preexisting brain damage. These problems should be considered in perioperative period.
authors.affiliation: Department of Anesthesiology, Yokohama City University School of Medicine.
authors.forename: K
authors.initials: K
authors.lastname: Kurahashi
authors.forename: T
authors.initials: T
authors.lastname: Andoh
authors.forename: K
authors.initials: K
authors.lastname: Sato
authors.forename: S
authors.initials: S
authors.lastname: Yamazaki
authors.forename: F
authors.initials: F
authors.lastname: Okumura
mesh_terms: D000293:Adolescent; D000768:Anesthesia, General / Q000379:methods*; D002331:Carnitine / Q000172:deficiency*; D004987:Ethers; D005260:Female; D006099:Granuloma / Q000601:surgery; D006801:Humans; D008738:Methyl Ethers; D009609:Nitrous Oxide; D000077149:Sevoflurane; D013577:Syndrome; D014133:Tracheal Diseases / Q000601:surgery; D014139:Tracheostomy; D014673:Vecuronium Bromide
chemical_list: D004987:Ethers; D008738:Methyl Ethers; D000077149:Sevoflurane; D014673:Vecuronium Bromide; D009609:Nitrous Oxide; D002331:Carnitine