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Pre-operative exercise and pyrexia as modifying factors in malignant hyperthermia (MH)

Riazi, Sheila ; Bersselaar, Luuk R.van den ; Islander, Gunilla LU ; Heytens, Luc ; Snoeck, Marc M.J. ; Bjorksten, Andrew ; Gillies, Robyn ; Dranitsaris, George ; Hellblom, Anna LU and Treves, Susan , et al. (2022) In Neuromuscular Disorders 32(8). p.628-634
Abstract

Malignant hyperthermia (MH) is a life-threatening reaction triggered by volatile anesthetics and succinylcholine. MH is caused by mutations in the skeletal muscle ryanodine receptor (RYR1) gene, as is rhabdomyolysis triggered by exertion and/or pyrexia. The discrepancy between the prevalence of risk genotypes and actual MH incidence remains unexplained. We investigated the role of pre-operative exercise and pyrexia as potential MH modifying factors. We included cases from 5 MH referral centers with 1) clinical features suggestive of MH, 2) confirmation of MH susceptibility on Contracture Testing (IVCT or CHCT) and/or RYR1 genetic testing, and a history of 3) strenuous exercise within 72 h and/or pyrexia >37.5 °C prior to the... (More)

Malignant hyperthermia (MH) is a life-threatening reaction triggered by volatile anesthetics and succinylcholine. MH is caused by mutations in the skeletal muscle ryanodine receptor (RYR1) gene, as is rhabdomyolysis triggered by exertion and/or pyrexia. The discrepancy between the prevalence of risk genotypes and actual MH incidence remains unexplained. We investigated the role of pre-operative exercise and pyrexia as potential MH modifying factors. We included cases from 5 MH referral centers with 1) clinical features suggestive of MH, 2) confirmation of MH susceptibility on Contracture Testing (IVCT or CHCT) and/or RYR1 genetic testing, and a history of 3) strenuous exercise within 72 h and/or pyrexia >37.5 °C prior to the triggering anesthetic. Characteristics of MH-triggering agents, surgery and succinylcholine use were collected. We identified 41 cases with general anesthesias resulting in an MH event (GA+MH, n = 41) within 72 h of strenuous exercise and/or pyrexia. We also identified previous general anesthesias without MH events (GA-MH, n = 51) in the index cases and their MH susceptible relatives. Apart from pre-operative exercise and/or pyrexia, trauma and acute abdomen as surgery indications, emergency surgery and succinylcholine use were also more common with GA+MH events. These observations suggest a link between pre-operative exercise, pyrexia and MH.

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organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
(Exertional) rhabdomyolysis, Malignant hyperthermia, RYR1
in
Neuromuscular Disorders
volume
32
issue
8
pages
7 pages
publisher
Elsevier
external identifiers
  • pmid:35738978
  • scopus:85132908875
ISSN
0960-8966
DOI
10.1016/j.nmd.2022.06.003
language
English
LU publication?
yes
id
a7a7e7ce-4fec-4bcb-9d60-3f034f7cb060
date added to LUP
2022-10-07 11:20:33
date last changed
2024-04-18 14:47:49
@article{a7a7e7ce-4fec-4bcb-9d60-3f034f7cb060,
  abstract     = {{<p>Malignant hyperthermia (MH) is a life-threatening reaction triggered by volatile anesthetics and succinylcholine. MH is caused by mutations in the skeletal muscle ryanodine receptor (RYR1) gene, as is rhabdomyolysis triggered by exertion and/or pyrexia. The discrepancy between the prevalence of risk genotypes and actual MH incidence remains unexplained. We investigated the role of pre-operative exercise and pyrexia as potential MH modifying factors. We included cases from 5 MH referral centers with 1) clinical features suggestive of MH, 2) confirmation of MH susceptibility on Contracture Testing (IVCT or CHCT) and/or RYR1 genetic testing, and a history of 3) strenuous exercise within 72 h and/or pyrexia &gt;37.5 °C prior to the triggering anesthetic. Characteristics of MH-triggering agents, surgery and succinylcholine use were collected. We identified 41 cases with general anesthesias resulting in an MH event (GA+MH, n = 41) within 72 h of strenuous exercise and/or pyrexia. We also identified previous general anesthesias without MH events (GA-MH, n = 51) in the index cases and their MH susceptible relatives. Apart from pre-operative exercise and/or pyrexia, trauma and acute abdomen as surgery indications, emergency surgery and succinylcholine use were also more common with GA+MH events. These observations suggest a link between pre-operative exercise, pyrexia and MH.</p>}},
  author       = {{Riazi, Sheila and Bersselaar, Luuk R.van den and Islander, Gunilla and Heytens, Luc and Snoeck, Marc M.J. and Bjorksten, Andrew and Gillies, Robyn and Dranitsaris, George and Hellblom, Anna and Treves, Susan and Kunst, Gudrun and Voermans, Nicol C. and Jungbluth, Heinz}},
  issn         = {{0960-8966}},
  keywords     = {{(Exertional) rhabdomyolysis; Malignant hyperthermia; RYR1}},
  language     = {{eng}},
  number       = {{8}},
  pages        = {{628--634}},
  publisher    = {{Elsevier}},
  series       = {{Neuromuscular Disorders}},
  title        = {{Pre-operative exercise and pyrexia as modifying factors in malignant hyperthermia (MH)}},
  url          = {{http://dx.doi.org/10.1016/j.nmd.2022.06.003}},
  doi          = {{10.1016/j.nmd.2022.06.003}},
  volume       = {{32}},
  year         = {{2022}},
}