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- W4319303916 abstract "La crise d’hyperthermie maligne (HM) est un évènement rare mais potentiellement mortel. Elle peut s’exprimer de façon variable chez les individus prédisposés génétiquement et exposés aux agents anesthésiques volatils et/ou au curare dépolarisant. Les signes classiques de la crise HM sont l’augmentation de production de C02 (FetCO2), la tachycardie, la rigidité musculaire, l’hyperthermie et la rhabdomyolyse, témoins de l’hypermétabolisme. L’incidence de la crise HM est estimée entre 1/15 000 et 1/75 000 anesthésies et sa mortalité serait proche de 5 %. La physiopathologie de l’HM repose sur une anomalie de l’homéostasie calcique du muscle squelettique. Lors de l’exposition aux agents déclenchants, le complexe de relâchement du calcium libère en excès le calcium dans le cytosol, responsable de la contracture musculaire observée. Ce complexe est constitué de deux canaux calciques, le récepteur de la ryanodine de type 1 (RyR1) et le récepteur des dihydropyridines (DPHR) et d’une protéine accessoire STAC3. Des mutations sur ces protéines peuvent être responsables d’HM. En cas de survenue d’une crise HM, la reconnaissance précoce des signes et la mise en œuvre de son traitement sont les éléments décisifs de la survie du patient. Le diagnostic de la sensibilité HM est réalisé soit par un bilan génétique, soit les tests de contracture in vitro halothane-caféine par biopsie musculaire. La physiopathologie, les mutations et leur caractère à pénétrance variable sont encore partiellement incompris. Les recommandations clés sont l’exclusion des agents déclenchants, la réflexion sur la prise en charge optimale préopératoire, per et post anesthésique des patients à risque ou sensibles HM. Malignant hyperthermia (MH) crisis is a rare but potentially fatal event. It can be expressed in individuals genetically predisposed and exposed to volatile anesthetic agents and/or depolarizing curare. The classic signs of the MH crisis are increased production of C02 (FetCO2), tachycardia, increased oxygen consumption, muscle rigidity, hyperthermia and rhabdomyolysis and witnesses of hypermetabolism. The incidence of the HM crisis is estimated between 1/15,000 to 1/75,000 anesthesias and its mortality is currently at 5%. The pathophysiology of MH is based on an anomaly of calcium homeostasis at the skeletal muscle level. Upon exposure to triggering agents, the calcium release complex (CRC) releases excess calcium into the cytosol, responsible for muscle contracture. This complex consists of two calcium channels, the ryanodine receptor type 1 (RyR1) and the dihydropyridine receptor (DPHR) and an accessory protein STAC3. Mutations in these proteins may be responsible for MH. In the event of an MH crisis, early recognition of the signs and the implementation of its treatment are the decisive elements of the patient's survival. Diagnosis of MH susceptibility is made either by genetic testing or in vitro halothane-caffeine contracture testing by muscle biopsy. The pathophysiology, the mutations and their variable penetrance character are still partially misunderstood. The key recommendations are the exclusion of triggering agents, and reflection on the optimal preoperative, per and post anesthetic management of patients at MH risk or at MH susceptibility." @default.
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- W4319303916 date "2023-03-01" @default.
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- W4319303916 title "Hyperthermie maligne de l’anesthésie" @default.
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- W4319303916 doi "https://doi.org/10.1016/j.anrea.2023.01.009" @default.
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