title: [Anesthetic management of a patient with adrenoleukodystrophy].
abstract: Adrenoleukodystrophy (ALD) is a genetic disorder with demyelination of the central nervous system and adrenal insufficiency. A 24-year-old man with ALD was scheduled for dental treatment under general anesthesia. He was diagnosted as having ALD at the age of 5. Past medical history included recurrent cervical cellulitis, adrenal insufficiency, mental retardation, muscle weakness and seizure disorder. General anesthesia was induced using betamethasone as a steroid cover, sevoflurane and nitrous oxide-oxygen and maintained with sevoflurane and nitrous oxide-oxygen. Nasal intubation was performed without using a muscle relaxant. Patients with ALD cannot metabolize very long chain fatty acid, so we did not use propofol containing long chain fatty acid. Operation and anesthesia were uneventful. There were no complications during and after anesthesia.
authors.affiliation: Sakai Special Needs Dental Clinic, Sakai 590-0801.
authors.forename: Maki
authors.initials: M
authors.lastname: Shinohara
authors.affiliation: Sakai Special Needs Dental Clinic, Sakai 590-0801.
authors.forename: Yohsuke
authors.initials: Y
authors.lastname: Hirose
authors.affiliation: Department of Dental Anesthesiology, Graduate School of Dentistry, Osaka University, Suita 565-0871.
authors.forename: Aiji
authors.initials: A
authors.lastname: Boku
authors.affiliation: Sakai Special Needs Dental Clinic, Sakai 590-0801.
authors.forename: Keiji Richard
authors.initials: KR
authors.lastname: Kimura
authors.affiliation: Department of Dental Anesthesiology, Graduate School of Dentistry, Osaka University, Suita 565-0871.
authors.forename: Yoshinari
authors.initials: Y
authors.lastname: Morimoto
authors.affiliation: Department of Dental Anesthesiology, Graduate School of Dentistry, Osaka University, Suita 565-0871.
authors.forename: Hitoshi
authors.initials: H
authors.lastname: Niwa
mesh_terms: D000074063:ATP Binding Cassette Transporter, Subfamily D, Member 1; D018528:ATP-Binding Cassette Transporters / Q000235:genetics / Q000502:physiology; D000308:Adrenocortical Hyperfunction; D000326:Adrenoleukodystrophy / Q000209:etiology; D000328:Adult; D000768:Anesthesia, General / Q000379:methods*; D003730:Dental Care for Persons with Disabilities / Q000379:methods*; D005231:Fatty Acids, Unsaturated / Q000378:metabolism; D020279:Hereditary Central Nervous System Demyelinating Diseases; D006801:Humans; D007442:Intubation, Intratracheal / Q000379:methods; D008297:Male; D008738:Methyl Ethers; D009609:Nitrous Oxide; D000077149:Sevoflurane; D055815:Young Adult
chemical_list: C115572:ABCD1 protein, human; D000074063:ATP Binding Cassette Transporter, Subfamily D, Member 1; D018528:ATP-Binding Cassette Transporters; D005231:Fatty Acids, Unsaturated; D008738:Methyl Ethers; D000077149:Sevoflurane; D009609:Nitrous Oxide