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Published on: October 23, 2019
[Anesthetic management of a patient with adrenoleukodystrophy]
Maki Shinohara1, Yohsuke Hirose1, Aiji Boku2
1Sakai Special Needs Dental Clinic, Sakai 590-0801.
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.
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