Metterlein, T. and Schuster, F. and Graf, B. M. and Anetseder, M. (2014) Malignant hyperthermia. ANAESTHESIST, 63 (12). pp. 908-918. ISSN 0003-2417, 1432-055X
Full text not available from this repository. (Request a copy)Abstract
Malignant hyperthermia (MH) is a rare hereditary, mostly subclinical myopathy. Trigger substances, such as volatile anesthetic agents and the depolarizing muscle relaxant succinylcholine can induce a potentially fatal metabolic increase in predisposed patients caused by a dysregulation of the myoplasmic calcium (Ca) concentration. Mutations in the dihydropyridine ryanodine receptor complex in combination with the trigger substances are responsible for an uncontrolled release of Ca from the sarcoplasmic reticulum. This leads to activation of the contractile apparatus and a massive increase in cellular energy production. Exhaustion of the cellular energy reserves ultimately results in local muscle cell destruction and subsequent cardiovascular failure. The clinical picture of MH episodes is very variable. Early symptoms are hypoxia, hypercapnia and cardiac arrhythmia whereas the body temperature rise, after which MH is named, often occurs later. Decisive for the course of MH episodes is a timely targeted therapy. Following introduction of the hydantoin derivative dantrolene, the previously high mortality of fulminant MH episodes could be reduced to well under 10 %. An MH predisposition can be detected using the invasive in vitro contracture test (IVCT) or mutation analysis. Few elaborate diagnostic procedures are in the developmental stage.
| Item Type: | Article |
|---|---|
| Uncontrolled Keywords: | CENTRAL CORE DISEASE; RYANODINE RECEPTOR GENE; CLINICAL GRADING SCALE; VITRO CONTRACTURE-TEST; SUSCEPTIBLE SWINE; CHROMOSOME 19Q12-13.2; CALCIUM-RELEASE; METABOLIC TEST; DELAYED-ONSET; RYR1 GENE; Intraoperative complications; Ryanodine receptor; Hypermetabolism; Mutation; Dantrolene |
| Subjects: | 600 Technology > 610 Medical sciences Medicine |
| Divisions: | Medicine > Lehrstuhl für Anästhesiologie |
| Depositing User: | Dr. Gernot Deinzer |
| Date Deposited: | 07 Aug 2019 09:12 |
| Last Modified: | 07 Aug 2019 09:12 |
| URI: | https://pred.uni-regensburg.de/id/eprint/9128 |
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