Pathophysiology and Treatment of Malignant Hyperthermia

Gregory, Haili; Weant, Kyle A.

doi:10.1097/tme.0000000000000344

Cited by 14 publications

(13 citation statements)

References 22 publications

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“…Masseter muscle rigidity, acidosis, and rising body temperature are also common. 2,4 In this case, the indolent rise in EtCO2 after induction that transformed into precipitously increasing EtCO2 levels triggered the call for help and allowed for prompt diagnosis. Hyperthermia, tachycardia, and hypotension followed shortly thereafter.…”

Section: Discussionmentioning

confidence: 94%

“…A mutation in ryanodine receptor gene (RYR1) is most commonly implicated, but the condition exhibits locus heterogeneity. 4 In MH, heat, lactate, and electrolyte imbalances from unabated muscular contractions causes acidosis, hypercapnia, tachycardia, hyperthermia, muscle rigidity, and rhabdomyolysis. 5 DS, the only pharmacological treatment for MH, antagonizes RyR1 receptors to inhibit sarcomeric calcium release.…”

Section: Discussionmentioning

confidence: 99%

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A rare case of late onset malignant hyperthermia

Rajan¹,

Victor²,

Kelly³

et al. 2022

Anaesth. pain intensive care

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A 42-year-old man with neck squamous cell carcinoma underwent awake fiberoptic intubation and tumor resection under general anesthesia. He developed malignant hyperthermia several hours into the surgical procedure. This case highlights malignant hyperthermia’s (MH) variable time course, pathognomonic signs, and the need for rapid diagnosis and treatment. Early recognition and treatment led to rapid resolution of MH. Ongoing discussion of MH is imperative because this disease is often difficult to diagnose early in its time course and may be fatal if not treated expeditiously. Abbreviations: DS - Dantrolene sodium; DSIS - dantrolene sodium injectable suspension; GA - general anesthesia; MH - malignant hyperthermia; EtCO2 - end tidal carbon dioxide; RYR1 - Ryanodine Receptor gene Key words: Anesthesia, General / methods; Dantrolene / therapeutic use; Diagnosis, Differential; Humans; Malignant Hyperthermia / diagnosis; Malignant Hyperthermia / etiology; Malignant Hyperthermia / physiopathology; Malignant Hyperthermia / therapy Citation: Victor R, Kelly R, Cockrill R, Katrivesis K, Nelson C, Rajan G. A rare case of late onset malignant hyperthermia. Anaesth. pain intensive care. 2022;26(4):546-550; DOI: 10.35975/apic.v26i4.1963 Received: November 19, 2021; Reviewed: April 25, 2022; Accepted: July 12. 2022

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Section: Discussionmentioning

confidence: 94%

Section: Discussionmentioning

confidence: 99%

A rare case of late onset malignant hyperthermia

Rajan¹,

Victor²,

Kelly³

et al. 2022

Anaesth. pain intensive care

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“…6 Treatment of MH requires timely termination of the anesthetic procedure, otherwise patients might suffer from systemic multi-organ failure, and eventually have an increased risk of death. 10 Dantrolene, a specific RyR1 antagonist, is currently the only FDA-approved drug available for MH. It relieves skeletal muscle contraction by inhibiting the release of calcium from the sarcoplasmic reticulum.…”

Section: Introductionmentioning

confidence: 99%

Epidemiological and clinical features of malignant hyperthermia: A scoping review

Cong,

Wan,

Wang

et al. 2023

Clinical Genetics

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Malignant hyperthermia (MH) is a potentially fatal inherited pharmacogenetic disorder related to pathogenic variants in the RYR1, CACNA1S, or STAC3 genes. Early recognition of the occurrence of MH and prompt medical treatment are indispensable to ensure a positive outcome. The purpose of this study was to provide valuable information for the early identification of MH by summarizing epidemiological and clinical features of MH. This scoping review followed the methodological framework recommended by Arksey and O'Malley. PubMed, Embase, and Web of science databases were searched for studies that evaluated the epidemical and clinical characteristics of MH. A total of 37 studies were included in this review, of which 26 were related to epidemiology and 24 were associated with clinical characteristics. The morbidity of MH varied from 0.18 per 100 000 to 3.9 per 100 000. The mortality was within the range of 0%–18.2%. Identified risk factors included sex, age, disorders associated with MH, and others. The most frequent initial clinical signs included hyperthermia, sinus tachycardia, and hypercarbia. The occurrence of certain signs, such as hypercapnia, delayed first temperature measurement, and peak temperature were associated with poor outcomes. The epidemiological and clinical features of MH varied considerably and some risk factors and typical clinical signs were identified. The main limitation of this review is that the treatment and management strategies were not assessed sufficiently due to limited information.

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“…The symptoms have quick onset, hereof, immediate diagnosis and proper treatment are crucial in preventing mortality. [4] The very first reports in the literature describing a condition that could correspond to MH were reported by several scientists on a single page in the JAMA in 1900. [5] However it wasn't until 1962, when the Denborough came up with the major breakthrough in discovering that MH is a genetic disorder.…”

Section: Introductionmentioning

confidence: 99%

Malignant hyperthermia – state of knowledge

Perszke

Egierska

Cieślak

et al. 2022

J Educ Health Sport

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Malignant hyperthermia (MH) is a relatively rare, but potentially lethal genetic disorder. That disease is characterized by hypermetabolic response of the skeletal muscles caused by exposure to triggering agents e.g. volatile anesthetics or depolarizing neuromuscular blockers. The object of this article was to review and assess the most recent published works about the epidemiology, etiology, pathomechanism, therapy of the MH and the new advances in all these fields. Authors scrutinized PubMed and Google Scholar using keywords: malignant hyperthermia, volatile anesthetics, ryanodine receptor mutation. In addition to this, the references of chosen articles were manually scoured for supplementary applicable articles. The literature was selected on the basis of a general medicine readership and prioritized clinical practice guidelines, systematic reviews and case reports. The references include the latest reports on malignant hyperthermia, apart from works of historical importance. Available treatment methods consist mainly of pharmacotherapy, symptomatic treatment and monitoring of vital parameters. Dantrolene is a first-choice drug in initial treatment of MH and is considered the only specific antidote. In spite of the constant advances in the areas of medicine there is still much to be discovered about MH. Further studies are necessary, as the amount of credible evidence is not adequate.

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Pathophysiology and Treatment of Malignant Hyperthermia

Cited by 14 publications

References 22 publications

A rare case of late onset malignant hyperthermia

A rare case of late onset malignant hyperthermia

Epidemiological and clinical features of malignant hyperthermia: A scoping review

Malignant hyperthermia – state of knowledge

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