Malignant hyperthermia

Kim, Dong-Chan
November 2012
Korean Journal of Anesthesiology;Nov2012, Vol. 63 Issue 5, p391
Academic Journal
Malignant hyperthermia (MH) is an uncommon, life-threatening pharmacogenetic disorder of the skeletal muscle. It presents as a hypermetabolic response in susceptible individuals to potent volatile anesthetics with/without depolarizing muscle relaxants; in rare cases, to stress from exertion or heat stress. Susceptibility to malignant hyperthermia (MHS) is inherited as an autosomally dominant trait with variable expression and incomplete penetrance. It is known that the pathophysiology of MH is related to an uncontrolled rise of myoplasmic calcium, which activates biochemical processes resulting in hypermetabolism of the skeletal muscle. In most cases, defects in the ryanodine receptor are responsible for the functional changes of calcium regulation in MH, and more than 300 mutations have been identified in the RYR1 gene, located on chromosome 19q13.1. The classic signs of MH include increase of end-tidal carbon dioxide, tachycardia, skeletal muscle rigidity, tachycardia, hyperthermia and acidosis. Up to now, muscle contracture test is regarded as the gold standard for the diagnosis of MHS though molecular genetic test is used, on a limited basis so far to diagnose MHS. The mortality of MH is dramatically decreased from 70-80% to less than 5%, due to an introduction of dantrolene sodium for treatment of MH, early detection of MH episode using capnography, and the introduction of diagnostic testing for MHS. This review summarizes the clinically essential and important knowledge of MH, and presents new developments in the field.


Related Articles

  • Metabolic effects as a cause of myotoxic effects of fluoroquinolones. Metterlein, Thomas; Schuster, Frank; Hager, Martin; Roewer, Norbert; Anetseder, Martin // Indian Journal of Pharmacology;Nov/Dec2015, Vol. 47 Issue 6, p616 

    Objectives: To investigate if fluoroquinolones (FQs) influence skeletal muscle metabolism of healthy and malignant hyperthermia susceptible (MHS) pigs. Materials and Methods: After approval from of the Animal Care Committee, 10 MHS pigs, and 6 MHS pigs were anesthetized with hemodynamic and...

  • Sevoflurane.  // Reactions Weekly;3/28/2009, Issue 1245, p33 

    The article describes the case of a man who developed malignant hyperthermia during treatment with sevoflurane for general anesthesia. The patient experienced symptoms such as tachycardia, hypertension and fever. Sevoflurane was discontinued to resolve his condition. Total IV anaesthesia was...

  • Malignant hyperthermia: a crisis response plan. Johns, Christopher D.; Staudt, Rebecca S.; Scholtis, Michael P.; Gavel, Theodore // OR Manager;Jun2012, Vol. 28 Issue 6, p18 

    The article presents information on hospital crisis management, with a focus on the appropriate medical response for patients with malignant hyperthermia (MH), a genetic skeletal muscle disorder incited by anesthesia drugs. Topics include the incidence of MH in U.S. hospitals; the mortality rate...

  • Endurance training enhances skeletal muscle interleukin-15 in human male subjects. Rinnov, Anders; Yfanti, Christina; Nielsen, Søren; Åkerström, Thorbjörn; Peijs, Lone; Zankari, Alaa; Fischer, Christian; Pedersen, Bente // Endocrine (1355008X);Mar2014, Vol. 45 Issue 2, p271 

    Regular endurance exercise promotes metabolic and oxidative changes in skeletal muscle. Overexpression of interleukin-15 (IL-15) in mice exerts similar metabolic changes in muscle as seen with endurance exercise. Muscular IL-15 production has been shown to increase in mice after weeks of regular...

  • Intracellular Ca2+-handling differs markedly between intact human muscle fibers and myotubes. Olsson, Karl; Cheng, Arthur J.; Alam, Seher; Al-Ameri, Mamdoh; Rullman, Eric; Westerblad, Håkan; Lanner, Johanna T.; Bruton, Joseph D.; Gustafsson, Thomas // Skeletal Muscle;Aug2015, Vol. 5 Issue 1, p1 

    Background: In skeletal muscle, intracellular Ca2+ is an important regulator of contraction as well as gene expression and metabolic processes. Because of the difficulties to obtain intact human muscle fibers, human myotubes have been extensively employed for studies of Ca2+-dependent processes...

  • Skeletal Muscle Function during Exercise--Fine-Tuning of Diverse Subsystems by Nitric Oxide. Suhr, Frank; Gehlert, Sebastian; Grau, Marijke; Bloch, Wilhelm // International Journal of Molecular Sciences;Apr2013, Vol. 14 Issue 4, p7109 

    Skeletal muscle is responsible for altered acute and chronic workload as induced by exercise. Skeletal muscle adaptations range from immediate change of contractility to structural adaptation to adjust the demanded performance capacities. These processes are regulated by mechanically and...

  • A novel atlas of gene expression in human skeletal muscle reveals molecular changes associated with aging. Jing Su; Ekman, Carl; Oskolkov, Nikolay; Lahti, Leo; Ström, Kristoffer; Brazma, Alvis; Groop, Leif; Rung, Johan; Hansson, Ola // Skeletal Muscle;10/9/2015, Vol. 5, p1 

    Background: Although high-throughput studies of gene expression have generated large amounts of data, most of which is freely available in public archives, the use of this valuable resource is limited by computational complications and non-homogenous annotation. To address these issues, we have...

  • Localized nuclear and perinuclear Ca2+ signals in intact mouse skeletal muscle fibers. Georgiev, Tihomir; Svirin, Mikhail; Jaimovich, Enrique; Fink, Rainer H. A.; Claflin, Dennis; Jayasinghe, Isuru // Frontiers in Physiology;Sep2015, p1 

    Nuclear Ca2+ is important for the regulation of several nuclear processes such as gene expression. Localized Ca2+ signals (LCSs) in skeletal muscle fibers of mice have been mainly studied as Ca2+ release events from the sarcoplasmic reticulum. Their location with regard to cell nuclei has not...

  • RNF14 is a regulator of mitochondrial and immune function in muscle. Ingham, Aaron B.; Osborne, Simone A.; Menzies, Moira; Briscoe, Suzie; Wei Chen; Kongsuwan, Kritaya; Reverter, Antonio; Jeanes, Angela; Dalrymple, Brian P.; Wijffels, Gene; Seymour, Robert; Hudson1, Nicholas J. // BMC Systems Biology;2014, Vol. 8 Issue 1, p1 

    Background Muscle development and remodelling, mitochondrial physiology and inflammation are thought to be inter-related and to have implications for metabolism in both health and disease. However, our understanding of their molecular control is incomplete. Results In this study we have...


Read the Article


Sorry, but this item is not currently available from your library.

Try another library?
Sign out of this library

Other Topics