Comparison Between the European and North American Protocols for Diagnosis of Malignant Hyperthermia Susceptibility in Humans

Islander, Gunilla; Twetman, Eva Ranklev

doi:10.1097/00132586-200004000-00040

Cited by 2 publications

(5 citation statements)

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“…The MHE category represents a "grey" area under permanent review and some MHE diagnoses most likely are normal variants 38 . This group contains both true positive and true negative results 39 and may also represent reduced susceptibility. There are MHE patients with the same mutation as MHS individuals in a family in which the proband died of MH 39 of replacing IVCT with a DNA-based test for susceptibility to MH, DNA and RNA samples from families with MHS members have been used for genetic screening.…”

Section: Discussionmentioning

confidence: 99%

“…This group contains both true positive and true negative results 39 and may also represent reduced susceptibility. There are MHE patients with the same mutation as MHS individuals in a family in which the proband died of MH 39 of replacing IVCT with a DNA-based test for susceptibility to MH, DNA and RNA samples from families with MHS members have been used for genetic screening. As a causative factor in MH, the ryanodine receptor gene (RYR1) has been the most widely studied and to date 15 mutations in the RYR1 gene which result in altered amino acid residues in the skeletal muscle ryanodine receptor have been shown to be causative of MH 41,42 .…”

Section: Discussionmentioning

confidence: 99%

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Suspected Malignant Hyperthermia Reactions in New Zealand

Pollock

Langton

Couchman

et al. 2002

Anaesth Intensive Care

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Early clinical signs, triggering agents, time to onset of reaction, mortality and methods of treatment were identified in 123 suspected malignant hyperthermia reactions. In vitro contracture test results were compared with clinical signs and the Malignant Hyperthermia Clinical Grading Scale. Increased end-tidal carbon dioxide is the earliest sign when not preceded by masseter spasm. Earlier diagnosis reduces the incidence of rigidity and severe metabolic acidosis. The combination of suxamethonium and a potent volatile anaesthetic agent triggers an earlier reaction compared with a volatile agent alone. There has been zero mortality since 1981, essentially due to a combination of advanced monitoring capability, increased anaesthetist awareness of malignant hyperthermia, and dantrolene availability. DNA analysis has identified nine New Zealand families with ryanodine receptor gene mutations. A positive DNA test indicates malignant hyperthermia susceptibility with "causative" mutations but discordance requires that negative DNA tests are confirmed with in vitro contracture test. This test also demonstrated the shortcomings of the Malignant Hyperthermia Clinical Grading Scale.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Suspected Malignant Hyperthermia Reactions in New Zealand

Pollock

Langton

Couchman

et al. 2002

Anaesth Intensive Care

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“…Testing is performed according to the European Malignant Hyperthermia Group (EMHG) procedure. 7 A muscle biopsy is performed under local anaesthetic and muscle strips are then attached to sensitive strain gauges. Each strip is exposed to various concentrations of halothane and caffeine.…”

Section: Discussionmentioning

confidence: 99%

“…The threshold is the concentration at which an increase in tension of at least 0.2 g is seen. 7 A patient can, on the basis of this test, be placed into one of three groups, according to the laboratory diagnosis.…”

Section: Discussionmentioning

confidence: 99%

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No response to trigger agents in a malignant hyperthermia‐susceptible patient

Claxton

Cross

Hopkins

2002

British Journal of Anaesthesia

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Malignant hyperthermia susceptibility is an inherited disorder, where a life-threatening condition can result from exposure to a trigger agent or agents. Succinylcholine and volatile anaesthetic agents are well established to be trigger agents in anaesthetic practice. We describe a case of a previously investigated malignant hyperthermia-susceptible patient who did not declare his status and was exposed to both succinylcholine and isoflurane, without any detectable reaction. Possible explanations for the lack of reaction include a subnormal temperature when exposed to isoflurane, and a significant interval between exposure to succinylcholine and isoflurane. Absence of a reaction to trigger agents on this occasion is not thought to indicate an incorrect diagnosis and labelling.

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Comparison Between the European and North American Protocols for Diagnosis of Malignant Hyperthermia Susceptibility in Humans

Cited by 2 publications

References 0 publications

Suspected Malignant Hyperthermia Reactions in New Zealand

Suspected Malignant Hyperthermia Reactions in New Zealand

No response to trigger agents in a malignant hyperthermia‐susceptible patient

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