悪性高熱症─最近の話題について─

• 向田 圭子

  広島県立障害者リハビリテーションセンター麻酔科

• 河本 昌志

  広島大学大学院医歯薬保健学研究院統合健康科学部門麻酔蘇生学

書誌事項

タイトル別名

• Malignant Hyperthermia -up-to-date topics-

抄録

  Malignant hyperthermia (MH) is an autosomal pharmacogenetic disease triggered by volatile halogenated anesthetics and/or depolarizing muscle relaxants. An MH reaction is life-threatening and is caused by an abnormally high release of myoplasmic Ca²⁺ from the sarcoplasmic reticulum through the ryanodine receptor 1 (RYR1). Mutations in the RYR1 gene are the major cause of MH and central core disease (CCD). More than 200 variants of RYR1 and 3 variants of α1-subunit of the voltage-dependent L-type Ca²⁺ channel (CACNA1S) linked to MH and CCD have been identified, but only 30 mutations are recognized as causative for MH and/or CCD. Myotubes cultured from MH patients showed increased sensitivity to RYR1 activators (caffeine, halothane and 4-chloro-m-cresol). Halothane leads to a significant contracture in MH susceptible (MHS) skeletal muscle bundles and increases the rate of calcium-induced calcium release (CICR) in MHS skinned fibers. More recently, store-operated Ca²⁺ entry was reported to be activated by halothane in human MHS skeletal skinned fibers. Dantrolene, the only specific treatment for MH, reduces the elevated myoplasmic Ca²⁺ level generated with MH reaction, but the exact mechanism underlying its inhibitory effect is unknown. For purging volatile anesthetics, modern anesthesia workstations require more time to flush with a high-flow fresh gas (O₂).

収録刊行物

• 日本臨床麻酔学会誌

  日本臨床麻酔学会誌 32 (5), 682-690, 2012

  日本臨床麻酔学会

キーワード

• 悪性高熱症
• リアノジン受容体
• ダントロレン

詳細情報

• CRID: 1390282679734517888
• NII論文ID: 10031074019
• NII書誌ID: AN00330159
• DOI: 10.2199/jjsca.32.682
• ISSN: 13499149
• ISSN: 02854945
• Web Site: https://search.jamas.or.jp/link/ui/2013034768
• 本文言語コード: ja
• データソース種別:
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• 抄録ライセンスフラグ: 使用不可