title: [Anesthetic management of a patient with carnitine palmitoyltransferase deficiency with a history of rhabdomyolysis].
abstract: Carnitine palmitoyltransferase (CPT) makes the fatty acids available through beta-oxidation. Deficiency of CPT causes difficulties of muscle cells to metabolize fatty acid. In affected patients, exercise, fast for a prolonged period, and stress, lead to exhaustion of the store of glucose in the body, and rhabdomyolysis may occur, since muscle can not utilize fatty acid as an alternative energy source. Therefore, anesthetic management of CPT deficiency needs infusion of glucose continuously, avoiding the use of the drugs that cause rhabdomyolysis and suppressing the surgical stress. A 67-year-old man, who had previous history of rhabdmyolysis during the postoperative period, and diagnosed CPT deficiency was scheduled for total gastrectomy. General anesthesia was induced with remifentanil, thiamylal and rocuronium after epidural catheter insertion. During surgery, general anesthesia was maintained with remifentanil, sevoflurane, and blood glucose was monitored frequently, with continuous glucose infusion. No complications occurred during anesthesia and perioperative course was uneventful.
authors.affiliation: Department of Anesthesiology, Kumamoto University Hospital, Kumamoto 860-8556.
authors.forename: Sayaka
authors.initials: S
authors.lastname: Nakamura
authors.forename: Michiko
authors.initials: M
authors.lastname: Sugita
authors.forename: Eriko
authors.initials: E
authors.lastname: Nakahara
authors.forename: Tatsuo
authors.initials: T
authors.lastname: Yamamoto
mesh_terms: D000368:Aged; D000768:Anesthesia, General / Q000379:methods*; D002334:Carnitine O-Palmitoyltransferase / Q000172:deficiency; D005743:Gastrectomy; D006801:Humans; D008297:Male; D008661:Metabolism, Inborn Errors / Q000150:complications*; D012206:Rhabdomyolysis / Q000150:complications*
chemical_list: D002334:Carnitine O-Palmitoyltransferase