Malignant hyperthermia: Report on a successful rescue of a case with the highest temperature of 44.2°C.

Autor: Lan H; Department of Anesthesiology, Lishui City People's Hospital, The Sixth Affiliated Hospital of Wenzhou Medical University, Lishui, Zhejiang, 323000, China., Duan G; Department of Anesthesiology, Lishui City People's Hospital, The Sixth Affiliated Hospital of Wenzhou Medical University, Lishui, Zhejiang, 323000, China., Zuo Y; Department of Anesthesiology, West China Hospital, Sichuan University, Chengdu, China., Lou T; Department of Anesthesiology, Lishui City People's Hospital, The Sixth Affiliated Hospital of Wenzhou Medical University, Lishui, Zhejiang, 323000, China., Xu J; Department of Anesthesiology, Lishui City People's Hospital, The Sixth Affiliated Hospital of Wenzhou Medical University, Lishui, Zhejiang, 323000, China., Shao C; Department of Anesthesiology, Lishui City People's Hospital, The Sixth Affiliated Hospital of Wenzhou Medical University, Lishui, Zhejiang, 323000, China., Wu J; Department of Anesthesiology, Lishui City People's Hospital, The Sixth Affiliated Hospital of Wenzhou Medical University, No. 15, Dazhong Street, Lishui, Zhejiang, 323000, China.
Jazyk: angličtina
Zdroj: Open medicine (Warsaw, Poland) [Open Med (Wars)] 2023 Oct 17; Vol. 18 (1), pp. 20230808. Date of Electronic Publication: 2023 Oct 17 (Print Publication: 2023).
DOI: 10.1515/med-2023-0808
Abstrakt: Malignant hyperthermia (MH) is an inherited skeletal muscle disorder caused primarily by a genetic mutation, usually in the calcium channel gene of the muscle. This mutation can lead to muscle hypersensitivity to volatile anesthetics (such as sevoflurane) and the depolarizing muscle relaxant succinylcholine, resulting in hyperthermia, muscle stiffness, metabolic disturbances, and other severe physiological reactions. This condition may prove fatal unless it is recognized in its early stages and treatment is administered promptly and aggressively. We report a 13-year-old adolescent who underwent laparoscopic appendectomy and developed MH after the use of inhalational anesthetics, manifested by unremitting hyperthermia with a maximum temperature of 44.2°C, muscle rigidity, tachycardia, hypercapnia; and malignant arrhythmias, cardiogenic shock, hyperkalemia, metabolic, and respiratory acidosis. After early and timely recognition, multidisciplinary management and administration of dantrolene, the case was successfully treated. Exome sequencing revealed a point mutation (amino acid change) on the RYR1 gene: c.12700G>C(p.Val4234Leu). Due to the lack of ready-made dantrolene in our hospital, the patient in this case received dantrolene treatment only 6 h after the first observation of high body temperature. We review the development of the disease and summarize the success of treatment and what can be done to improve the chances of saving the patient's life if dantrolene is not available in time.
Competing Interests: Conflict of interest: The authors declare that they have no conflict of interest.
(© 2023 the author(s), published by De Gruyter.)
Databáze: MEDLINE