title: [Anesthetic management in a patient with trifunctional protein deficiency].
abstract: A 4-year-old girl with trifunctional protein deficiency was scheduled for gastrostomy. She had recurrent episodes of rhabdomyolysis triggered by fasting, infection, stress and uncertain causes. In the management of anesthesia, we avoided both propofol and inhalational anesthetics and anesthetized her with benzodiazepine and opioid combined with regional anesthesia to minimize the stress response. Her metabolism was kept stable throughout the intraoperative period. However, on the second postoperative day, she developed rhabdomyolysis and was treated with glucose containing infusion. This case provides instructive information that strict management is needed for patients with trifunctional protein deficiency in the perioperative period.
authors.affiliation: Department of Anesthesia, Odawara City Hospital, Odawara 250-8558.
authors.forename: Kyoko
authors.initials: K
authors.lastname: Makise
authors.forename: Gaku
authors.initials: G
authors.lastname: Inagawa
authors.forename: Koui
authors.initials: K
authors.lastname: Ka
mesh_terms: D015094:3-Hydroxyacyl CoA Dehydrogenases / Q000172:deficiency*; D000102:Acetyl-CoA C-Acyltransferase / Q000172:deficiency*; D000701:Analgesics, Opioid; D000758:Anesthesia; D001569:Benzodiazepines; D002675:Child, Preschool; D004746:Enoyl-CoA Hydratase / Q000172:deficiency*; D005260:Female; D005774:Gastrostomy; D006801:Humans; D008052:Lipid Metabolism, Inborn Errors / Q000150:complications; D063989:Long-Chain-3-Hydroxyacyl-CoA Dehydrogenase; D063991:Mitochondrial Trifunctional Protein; D009097:Multienzyme Complexes / Q000172:deficiency*; D012008:Recurrence; D012206:Rhabdomyolysis / Q000209:etiology
chemical_list: D000701:Analgesics, Opioid; D009097:Multienzyme Complexes; D001569:Benzodiazepines; D015094:3-Hydroxyacyl CoA Dehydrogenases; D063989:Long-Chain-3-Hydroxyacyl-CoA Dehydrogenase; D000102:Acetyl-CoA C-Acyltransferase; D063991:Mitochondrial Trifunctional Protein; D004746:Enoyl-CoA Hydratase