[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: Sayaka Nakamura, Michiko Sugita, Eriko Nakahara, Tatsuo Yamamoto
• Journal: Masui. The Japanese journal of anesthesiology (Masui) Vol. 62 Issue 3 Pg. 354-7 (Mar 2013) ISSN: 0021-4892 [Print] Japan
• PMID: 23544345 (Publication Type: Case Reports, English Abstract, Journal Article)

Chemical References

• Carnitine O-Palmitoyltransferase

Topics

• Aged
• Anesthesia, General (methods)
• Carnitine O-Palmitoyltransferase (deficiency)
• Gastrectomy
• Humans
• Male
• Metabolism, Inborn Errors (complications)
• Rhabdomyolysis (complications)