Consensus Statement of the Malignant Hyperthermia Association of the United States on Unresolved Clinical Questions Concerning the Management of Patients With Malignant Hyperthermia

Litman, Ronald S.; Smith, Victoria I.; Larach, Marilyn Green; Mayes, Lena M.; Shukry, Mohanad; Theroux, Mary C.; Watt, Stacey; Wong, Cynthia A.

doi:10.1213/ane.0000000000004039

Cited by 28 publications

(16 citation statements)

References 56 publications

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“…The mechanism of dantrolene is to act as an antagonist, and it binds to a specific region in the ryanodine receptor 1 (RYR-1) channel. It reduces the uncontrolled release of intracellular calcium and should be available anywhere when general anesthesia is performed [ 1 , 8 ].…”

Section: Discussionmentioning

confidence: 99%

Isoflurane Induced Malignant Hyperthermia in a Patient with Glucose 6-Phosphate Dehydrogenase Deficiency and Growth Hormone Abuse

Ravaei

Barhaghtalab

Salehi

et al. 2020

Case Reports in Anesthesiology

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Malignant hyperthermia is a pharmacogenetic disorder in the regulation of calcium in skeletal muscles which is related to an uninhibited muscle hypermetabolic reaction to potent inhalation agents, the depolarizing muscle relaxant succinylcholine, and to stressors such as vigorous exercise and heat. MH is diagnosed by the clinical presentation of the disease and laboratory testing. There are a few previous studies working on if there is an association between the occurrence of malignant hyperthermia and the existence of glucose 6-phosphate dehydrogenase (G6PD) deficiency, and there was no report on growth hormone doping in the literature. So, our main goal was to show this rare case of malignant hyperthermia seen in a G6PD patient with growth hormone abuse who underwent surgery and to find if there is an association between G6PD deficiency, growth hormone abuse, and malignant hyperthermia. Our patient was a 17-year-old boy with right lower quadrant abdominal pain and tenderness who underwent appendectomy. At the end of the operation, the patient developed with an increased heart rate (sinus tachycardia), increased body temperature and end-tidal carbon dioxide (ETCO2) level, masseter muscle rigidity, and then, generalized body rigidity, so the malignant hyperthermia susceptibility was considered. The patient was managed by cooling down the patient and the administration of dantrolene. We could hypothesize that malignant hyperthermia might be associated with G6PD deficiency as a triggering factor, but has no association with recombinant human growth hormone (rhGH) abuse. Another main lesson which this study tells us is to make a careful and proper history taking before going on an operation for preoperative evaluation and identification of patients with any form of suspicious drug abuse in order not to receive volatile inhalational agents and, also, performing some preventive measures including avoidance of heat extremes and restricting athletic activity in a patient with a history of malignant hyperthermia, and if the malignant hyperthermia susceptibility is suspected, urgent management should be carried out. As the association between G6PD deficiency, human growth hormone abuse, and malignant hyperthermia has remained unclear up-to-date, further potent studies are seriously needed in the future.

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Section: Discussionmentioning

confidence: 99%

Isoflurane Induced Malignant Hyperthermia in a Patient with Glucose 6-Phosphate Dehydrogenase Deficiency and Growth Hormone Abuse

Ravaei

Barhaghtalab

Salehi

et al. 2020

Case Reports in Anesthesiology

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“…As MH crises are mostly limited to the clinical setting, therapeutic efforts concentrate on their avoidance by testing patients potentially at risk either functionally, with a caffeine-sensitivity test, or genetically for known disease-causing mutations. MH crisis management is accomplished by whole-body cooling and the rapid administration of the MH antidote dantrolene [ 51 ].…”

Section: Normokalemic Periodic Paralysis (Normopp)mentioning

confidence: 99%

Skeletal muscle CaV1.1 channelopathies

Flucher

2020

Pflugers Arch - Eur J Physiol

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Ca V 1.1 is specifically expressed in skeletal muscle where it functions as voltage sensor of skeletal muscle excitation-contraction (EC) coupling independently of its functions as L-type calcium channel. Consequently, all known Ca V 1.1-related diseases are muscle diseases and the molecular and cellular disease mechanisms relate to the dual functions of Ca V 1.1 in this tissue. To date, four types of muscle diseases are known that can be linked to mutations in the CACNA1S gene or to splicing defects. These are hypo-and normokalemic periodic paralysis, malignant hyperthermia susceptibility, Ca V 1.1-related myopathies, and myotonic dystrophy type 1. In addition, the Ca V 1.1 function in EC coupling is perturbed in Native American myopathy, arising from mutations in the Ca V 1.1-associated protein STAC3. Here, we first address general considerations concerning the possible roles of Ca V 1.1 in disease and then discuss the state of the art regarding the pathophysiology of the Ca V 1.1-related skeletal muscle diseases with an emphasis on molecular disease mechanisms.

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“…31 32 Patients with a history of a non-anaesthesia-related malignant hyperthermia–like illness should therefore be considered on a case-by-case basis, in order to assess the likelihood of malignant hyperthermia susceptibility and the indication for a referral for in vitro contracture test (or caffeine halothane contraction test) or genetic testing. 33 34…”

Section: Malignant Hyperthermiamentioning

confidence: 99%

Anaesthesia and neuromuscular disorders: what a neurologist needs to know

et al. 2020

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Neurologists are often asked for specific advice regarding patients with neuromuscular disease who require general anaesthesia. However, guidelines on specific neuromuscular disorders do not usually include specific guidelines or pragmatic advice regarding (regional and/or general) anaesthesia or procedural sedation. Furthermore, the medical literature on this subject is mostly limited to publications in anaesthesiology journals. We therefore summarise general recommendations and specific advice for anaesthesia in different neuromuscular disorders to provide a comprehensive and accessible overview of the knowledge on this topic essential for clinical neurologists. A preoperative multidisciplinary approach involving anaesthesiologists, cardiologists, chest physicians, surgeons and neurologists is crucial. Depolarising muscle relaxants (succinylcholine) should be avoided at all times. The dose of non-depolarising muscle relaxants must be reduced and their effect monitored. Patients with specific mutations in RYR1 (ryanodine receptor 1) and less frequently in CACNA1S (calcium channel, voltage-dependent, L type, alpha 1S subunit) and STAC3 (SH3 and cysteine rich domain 3) are at risk of developing a life-threatening malignant hyperthermia reaction.

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Consensus Statement of the Malignant Hyperthermia Association of the United States on Unresolved Clinical Questions Concerning the Management of Patients With Malignant Hyperthermia

Cited by 28 publications

References 56 publications

Isoflurane Induced Malignant Hyperthermia in a Patient with Glucose 6-Phosphate Dehydrogenase Deficiency and Growth Hormone Abuse

Isoflurane Induced Malignant Hyperthermia in a Patient with Glucose 6-Phosphate Dehydrogenase Deficiency and Growth Hormone Abuse

Skeletal muscle CaV1.1 channelopathies

Anaesthesia and neuromuscular disorders: what a neurologist needs to know

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