{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,15]],"date-time":"2026-10-15T12:01:52Z","timestamp":1792065712760,"version":"4.4.0"},"reference-count":19,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2024,4,22]],"date-time":"2024-04-22T00:00:00Z","timestamp":1713744000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2024,4,22]],"date-time":"2024-04-22T00:00:00Z","timestamp":1713744000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["JA Clin Rep"],"abstract":"<jats:title>Abstract<\/jats:title><jats:sec>\n                <jats:title>Background<\/jats:title>\n                <jats:p>Malignant hyperthermia (MH) is a rare, life-threatening disorder of calcium homeostasis in skeletal muscle cells that is triggered by volatile anesthetics and succinylcholine, leading to a hypermetabolic reaction. The pathogenic ryanodine receptor 1 (RYR1) gene variant is critical. Patients susceptible to MH should avoid triggering agents, and total intravenous anesthesia (TIVA) is preferred. Remimazolam is safe in patients with suspected MH.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Case presentation<\/jats:title>\n                <jats:p>We present the first case of remimazolam treatment in a genetically confirmed patient with MH without MH development. A 72-year-old man with a family history of MH underwent remimazolam-based TIVA. After informed consent was obtained, a muscle biopsy and genetic testing were performed. Intraoperatively and postoperatively, the patient exhibited no signs of MH. An enhanced function of the RYR1 channel into releasing calcium was indicated, and the genetic testing revealed a pathogenic variant of <jats:italic>RYR1<\/jats:italic>.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Conclusions<\/jats:title>\n                <jats:p>Remimazolam-based TIVA is safe in patients confirming the diagnosis of MH.<\/jats:p>\n              <\/jats:sec>","DOI":"10.1186\/s40981-024-00710-7","type":"journal-article","created":{"date-parts":[[2024,4,22]],"date-time":"2024-04-22T08:02:03Z","timestamp":1713772923000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Remimazolam-based total intravenous anesthesia in a patient with a confirmed diagnosis of malignant hyperthermia: a case report"],"prefix":"10.1186","volume":"10","author":[{"given":"Hiroshi","family":"Kondo","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Keiko","family":"Mukaida","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kurumi","family":"Sasai","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yukiko","family":"Nao","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ken","family":"Hashimoto","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hirotsugu","family":"Miyoshi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rieko","family":"Kanzaki","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1997-082X","authenticated-orcid":false,"given":"Yasuo M.","family":"Tsutsumi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2024,4,22]]},"reference":[{"key":"710_CR1","doi-asserted-by":"publisher","first-page":"709","DOI":"10.1007\/s12630-018-1108-0","volume":"65","author":"S Riazi","year":"2018","unstructured":"Riazi S, Kraeva N, Hopkins PM. Updated guide for the management of malignant hyperthermia. Can J Anaesth. 2018;65:709\u201321. https:\/\/doi.org\/10.1007\/s12630-018-1108-0.","journal-title":"Can J Anaesth"},{"key":"710_CR2","doi-asserted-by":"publisher","first-page":"4087","DOI":"10.1093\/hmg\/ddac145","volume":"31","author":"JJ Johnston","year":"2022","unstructured":"Johnston JJ, Dirksen RT, Girard T, Hopkins PM, Kraeva N, Ognoon M, et al. Updated variant curation expert panel criteria and pathogenicity classifications for 251 variants for RYR1-related malignant hyperthermia susceptibility. Hum Mol Genet. 2022;31:4087\u201393. https:\/\/doi.org\/10.1093\/hmg\/ddac145.","journal-title":"Hum Mol Genet"},{"key":"710_CR3","doi-asserted-by":"publisher","first-page":"3869","DOI":"10.3390\/jcm12123869","volume":"12","author":"L Frassanito","year":"2023","unstructured":"Frassanito L, Sbaraglia F, Piersanti A, Vassalli F, Lucente M, Filetici N, et al. Real evidence and misconceptions about malignant hyperthermia in children: a narrative review. J Clin Med. 2023;12:3869. https:\/\/doi.org\/10.3390\/jcm12123869.","journal-title":"J Clin Med"},{"key":"710_CR4","doi-asserted-by":"publisher","first-page":"655","DOI":"10.1111\/anae.15317","volume":"76","author":"PM Hopkins","year":"2021","unstructured":"Hopkins PM, Girard T, Dalay S, Jenkins B, Thacker A, Patteril M, et al. Malignant hyperthermia 2020: guideline from the Association of Anaesthetists. Anaesthesia. 2021;76:655\u201364. https:\/\/doi.org\/10.1111\/anae.15317.","journal-title":"Anaesthesia"},{"key":"710_CR5","doi-asserted-by":"publisher","first-page":"120","DOI":"10.1016\/j.bja.2020.09.029","volume":"126","author":"H R\u00fcffert","year":"2021","unstructured":"R\u00fcffert H, Bastian B, Bendixen D, Girard T, Heiderich S, Hellblom A, et al. Consensus guidelines on perioperative management of malignant hyperthermia suspected or susceptible patients from the European Malignant Hyperthermia Group. Br J Anaesth. 2021;126:120\u201330. https:\/\/doi.org\/10.1016\/j.bja.2020.09.029.","journal-title":"Br J Anaesth"},{"key":"710_CR6","doi-asserted-by":"publisher","first-page":"335","DOI":"10.1007\/s00540-023-03186-4","volume":"37","author":"K Hirota","year":"2023","unstructured":"Hirota K. Remimazolam: a new string to the TIVA bow. J Anesth. 2023;37:335\u20139. https:\/\/doi.org\/10.1007\/s00540-023-03186-4.","journal-title":"J Anesth"},{"key":"710_CR7","doi-asserted-by":"publisher","first-page":"8845129","DOI":"10.1155\/2021\/8845129","volume":"2021","author":"T Watanabe","year":"2021","unstructured":"Watanabe T, Miyoshi H, Noda Y, Narasaki S, Morio A, Toyota Y, et al. Effects of remimazolam and propofol on Ca(2+) regulation by ryanodine receptor 1 with malignant hyperthermia mutation. 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Eur J Anaesthesiol. 2023;40:841\u201353. https:\/\/doi.org\/10.1097\/EJA.0000000000001902.","journal-title":"Eur J Anaesthesiol"},{"key":"710_CR16","doi-asserted-by":"publisher","first-page":"3445","DOI":"10.1038\/s41598-021-82024-7","volume":"11","author":"K Hoppe","year":"2021","unstructured":"Hoppe K, Jurkat-Rott K, Kranepuhl S, Wearing S, Heiderich S, Merlak S, et al. Relevance of pathogenicity prediction tools in human RYR1 variants of unknown significance. Sci Rep. 2021;11:3445. https:\/\/doi.org\/10.1038\/s41598-021-82024-7.","journal-title":"Sci Rep"},{"key":"710_CR17","doi-asserted-by":"publisher","first-page":"1288","DOI":"10.1038\/s41436-021-01125-w","volume":"23","author":"JJ Johnston","year":"2021","unstructured":"Johnston JJ, Dirksen RT, Girard T, Gonsalves SG, Hopkins PM, Riazi S, et al. Variant curation expert panel recommendations for RYR1 pathogenicity classifications in malignant hyperthermia susceptibility. 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Neuromuscul Disord. 2023;33:951\u201363. https:\/\/doi.org\/10.1016\/j.nmd.2023.10.019.","journal-title":"Neuromuscul Disord"}],"container-title":["JA Clinical Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s40981-024-00710-7.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1186\/s40981-024-00710-7\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s40981-024-00710-7.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,4,22]],"date-time":"2024-04-22T08:06:35Z","timestamp":1713773195000},"score":1,"resource":{"primary":{"URL":"https:\/\/jaclinicalreports.springeropen.com\/articles\/10.1186\/s40981-024-00710-7"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,4,22]]},"references-count":19,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2024,12]]}},"alternative-id":["710"],"URL":"https:\/\/doi.org\/10.1186\/s40981-024-00710-7","relation":{},"ISSN":["2363-9024"],"issn-type":[{"value":"2363-9024","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,4,22]]},"assertion":[{"value":"25 March 2024","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"8 April 2024","order":2,"name":"revised","label":"Revised","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"11 April 2024","order":3,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"22 April 2024","order":4,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"E2015-9151\u201318 (Sept. 21, 2022, Hiroshima University).","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"Written informed consent was obtained from the patient for the publication of this case report and the accompanying images. 2022\u201356 (Mar. 20, 2023. National Hospital Organization, Higashishima Medical Center).","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"The authors declare that they have no competing interests.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"26"}}