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Review
. 2022 Nov 4;9(11):1692.
doi: 10.3390/children9111692.

Malignant Hyperthermia in PICU-From Diagnosis to Treatment in the Light of Up-to-Date Knowledge

Martina Klincová  1   2 , Dagmar Štěpánková  1   2   3 , Ivana Schröderová  1   4 , Eva Klabusayová  2 , Petr Štourač  1   2   3
Affiliations
Review

Malignant Hyperthermia in PICU-From Diagnosis to Treatment in the Light of Up-to-Date Knowledge

Martina Klincová et al. Children (Basel). .

Abstract

Malignant Hyperthermia (MH) is a rare, hereditary, life-threatening disease triggered by volatile anesthetics and succinylcholine. Rarely, MH can occur after non-pharmacological triggers too. MH was detected more often in children and young adults, which makes this topic very important for every pediatric specialist, both anesthesiologists and intensivists. MH crisis is a life-threatening severe hypermetabolic whole-body reaction. Triggers of MH are used in pediatric intensive care unit (PICU) as well, volatile anesthetics in difficult sedation, status asthmaticus or epilepticus, and succinylcholine still sometimes in airway management. Recrudescence or delayed onset of MH crisis hours after anesthesia was previously described. MH can also be a cause of rhabdomyolysis and hyperpyrexia in the PICU. In addition, patients with neuromuscular diseases are often admitted to PICU and they might be at risk for MH. The most typical symptoms of MH are hypercapnia, tachycardia, hyperthermia, and muscle rigidity. Thinking of the MH as the possible cause of deterioration of a patient's clinical condition is the key to early diagnosis and treatment. The sooner the correct treatment is commenced, the better patient´s outcome. This narrative review article aims to summarize current knowledge and guidelines about recognition, treatment, and further management of MH in PICU.

Keywords: PICU; RYR1; children; cooling; critical; dantrolene; malignant hyperthermia; sevoflurane; succinylcholine; triggers.

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Conflict of interest statement

The authors declare no conflict of interest. The funders had no role in the design of the study; in the collection, analyses, or interpretation of data; in the writing of the manuscript, or in the decision to publish the results.

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References

    1. Rosenberg H., Pollock N., Schiemann A., Bulger T., Stowell K. Malignant hyperthermia: A review. Orphanet. J. Rare. Dis. 2015;10:93. doi: 10.1186/s13023-015-0310-1. - DOI - PMC - PubMed
    1. Robinson R., Carpenter D., Shaw M.-A., Halsall J., Hopkins P. Mutations in RYR1 in malignant hyperthermia and central core disease. Hum. Mutat. 2006;27:977–989. doi: 10.1002/humu.20356. - DOI - PubMed
    1. Carpenter D., Ringrose C., Leo V., Morris A., Robinson R.L., Halsall P.J., Hopkins P.M., Shaw M.-A. The role of CACNA1S in predisposition to malignant hyperthermia. BMC Med. Genet. 2009;10:104. doi: 10.1186/1471-2350-10-104. - DOI - PMC - PubMed
    1. Zaharieva I.T., Sarkozy A., Munot P., Manzur A., O’Grady G., Rendu J., Malfatti E., Amthor H., Servais L., Urtizberea J.A., et al. STAC3 variants cause a congenital myopathy with distinctive dysmorphic features and malignant hyperthermia susceptibility. Hum. Mutat. 2018;39:1980–1994. doi: 10.1002/humu.23635. - DOI - PubMed
    1. European Malignant Hyperthermia Group STAC3 Variants in Susceptibility to Malignant Hyperthermia. [(accessed on 21 December 2021)]. Available online: https://www.emhg.org/stac3.

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