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Anesthesiology Clinics Mar 2020Malignant hyperthermia (MH) is a rare but potentially lethal skeletal muscle disorder affecting calcium release channels. It is inherited in a mendelian autosomal... (Review)
Review
Malignant hyperthermia (MH) is a rare but potentially lethal skeletal muscle disorder affecting calcium release channels. It is inherited in a mendelian autosomal dominant pattern with variable penetration. The initial clinical manifestations are of a hypermetabolic state with increased CO2 production, respiratory acidosis, increased temperature, and increased oxygen demands. If diagnosed late, MH progresses to multi-organ system failure and death. Current data suggest that mortality has improved to less than 5%. The gold standard for ruling out MH is the contracture test. Genetic testing is also available. MH-susceptible individuals should be clearly identified for safe administration of future anesthetics.
Topics: Anesthesia; Humans; Malignant Hyperthermia
PubMed: 32008650
DOI: 10.1016/j.anclin.2019.10.010 -
British Journal of Anaesthesia Jan 2021Malignant hyperthermia is a potentially fatal condition, in which genetically predisposed individuals develop a hypermetabolic reaction to potent inhalation anaesthetics...
Malignant hyperthermia is a potentially fatal condition, in which genetically predisposed individuals develop a hypermetabolic reaction to potent inhalation anaesthetics or succinylcholine. Because of the rarity of malignant hyperthermia and ethical limitations, there is no evidence from interventional trials to inform the optimal perioperative management of patients known or suspected with malignant hyperthermia who present for surgery. Furthermore, as the concentrations of residual volatile anaesthetics that might trigger a malignant hyperthermia crisis are unknown and manufacturers' instructions differ considerably, there are uncertainties about how individual anaesthetic machines or workstations need to be prepared to avoid inadvertent exposure of susceptible patients to trigger anaesthetic drugs. The present guidelines are intended to bundle the available knowledge about perioperative management of malignant hyperthermia-susceptible patients and the preparation of anaesthesia workstations. The latter aspect includes guidance on the use of activated charcoal filters. The guidelines were developed by members of the European Malignant Hyperthermia Group, and they are based on evaluation of the available literature and a formal consensus process. The most crucial recommendation is that malignant hyperthermia-susceptible patients should receive anaesthesia that is free of triggering agents. Providing that this can be achieved, other key recommendations include avoidance of prophylactic administration of dantrolene; that preoperative management, intraoperative monitoring, and care in the PACU are unaltered by malignant hyperthermia susceptibility; and that malignant hyperthermia patients may be anaesthetised in an outpatient setting.
Topics: Anesthesia; Consensus; Europe; Humans; Malignant Hyperthermia; Perioperative Care
PubMed: 33131754
DOI: 10.1016/j.bja.2020.09.029 -
Expert Opinion on Drug Safety Sep 2021: Many drugs are known to induce malignant syndromes. The most common malignant syndromes are induced by the use of antipsychotics and anesthetics and the withdrawal of... (Review)
Review
: Many drugs are known to induce malignant syndromes. The most common malignant syndromes are induced by the use of antipsychotics and anesthetics and the withdrawal of anti-Parkinson drugs. As the clinical manifestations of antipsychotic malignant syndrome, Parkinson's disease hyperpyrexia syndrome and anesthetic-induced malignant syndrome are very similar, they are easily confused in the clinic.: We reviewed articles published between 1960 and April 2021 describing malignant syndromes. This paper provides a detailed literature review of malignant syndromes and important guidance for the diagnosis and treatment of malignant syndromes to clinicians.: Although malignant syndromes are rare conditions with a low incidence, these conditions usually progress rapidly and can endanger patients' lives, meriting attention from clinicians. The typical clinical manifestations of malignant syndromes are hyperpyrexia, muscular rigidity, an altered mental status and increased levels of creatine kinase; however, the pathophysiology, treatment and prognosis of different malignant syndromes are quite different. Prompt diagnosis and treatment may significantly improve the prognosis of patients with malignant syndromes.
Topics: Anesthetics; Antiparkinson Agents; Antipsychotic Agents; Humans; Incidence; Malignant Hyperthermia; Neuroleptic Malignant Syndrome; Parkinson Disease; Prognosis
PubMed: 33866917
DOI: 10.1080/14740338.2021.1919622 -
The Israel Medical Association Journal... Apr 2023
Topics: Humans; Malignant Hyperthermia
PubMed: 37129134
DOI: No ID Found -
Human Molecular Genetics Nov 2022The ClinGen malignant hyperthermia susceptibility (MHS) variant curation expert panel specified the American College of Medical Genetics and Genomics/Association of...
The ClinGen malignant hyperthermia susceptibility (MHS) variant curation expert panel specified the American College of Medical Genetics and Genomics/Association of Molecular Pathologists (ACMG/AMP) criteria for RYR1-related MHS and a pilot analysis of 84 variants was published. We have now classified an additional 251 variants for RYR1-related MHS according to current ClinGen standards and updated the criteria where necessary. Criterion PS4 was modified such that individuals with multiple RYR1 variants classified as pathogenic (P), likely pathogenic (LP), or variant of uncertain significance (VUS) were not considered as providing evidence for pathogenicity. Criteria PS1 and PM5 were revised to consider LP variants at the same amino-acid residue as providing evidence for pathogenicity at reduced strength. Finally, PM1 was revised such that if PS1 or PM5 are used PM1, if applicable, should be downgraded to supporting. Of the 251 RYR1 variants, 42 were classified as P/LP, 16 as B/LB, and 193 as VUS. The primary driver of 175 VUS classifications was insufficient evidence supporting pathogenicity, rather than evidence against pathogenicity. Functional data supporting PS3/BS3 was identified for only 13 variants. Based on the posterior probabilities of pathogenicity and variant frequencies in gnomAD, we estimated the prevalence of individuals with RYR1-related MHS pathogenic variants to be between 1/300 and 1/1075, considerably higher than current estimates. We have updated ACMG/AMP criteria for RYR1-related MHS and classified 251 variants. We suggest that prioritization of functional studies is needed to resolve the large number of VUS classifications and allow for appropriate risk assessment. RYR1-related MHS pathogenic variants are likely to be more common than currently appreciated.
Topics: Humans; Genetic Testing; Genetic Variation; Malignant Hyperthermia; Ryanodine Receptor Calcium Release Channel; United States; Virulence
PubMed: 35849058
DOI: 10.1093/hmg/ddac145 -
Ugeskrift For Laeger Mar 2023Hyperthermia is a severe complication to intake of methamphetamines due to generalised overactivation of metabolism and muscle activity combined with vasoconstriction....
Hyperthermia is a severe complication to intake of methamphetamines due to generalised overactivation of metabolism and muscle activity combined with vasoconstriction. In this case report, a patient presented to the emergency department after injection of 2 g "crystal meth", and advanced into fatal hyperthermia and organ failure in the intensive care unit. Treatment of substance-induced hyperthermia is symptomatic and reducing metabolism with benzodiazepines and actively lowering body temperature with ice packs and cold intravenous fluids are appropriate interventions. Dantrolene may be used but is still to be properly investigated.
Topics: Humans; Methamphetamine; Fatal Outcome; Malignant Hyperthermia; Hyperthermia, Induced
PubMed: 36999287
DOI: No ID Found -
Brazilian Journal of Anesthesiology... 2023
Topics: Humans; Malignant Hyperthermia
PubMed: 36963956
DOI: 10.1016/j.bjane.2023.03.001 -
CMAJ : Canadian Medical Association... Jun 2022
Topics: Humans; Malignant Hyperthermia; Rhabdomyolysis; Succinylcholine
PubMed: 35760427
DOI: 10.1503/cmaj.146480-l -
Journal of Perianesthesia Nursing :... Oct 2018Malignant hyperthermia is an uncommon, but potentially lethal condition that may be encountered during the perioperative period. There is wide variability in the manner... (Review)
Review
Malignant hyperthermia is an uncommon, but potentially lethal condition that may be encountered during the perioperative period. There is wide variability in the manner in which malignant hyperthermia may manifest. For a patient to survive a malignant hyperthermia crisis, prompt recognition and treatment is of paramount importance. Perioperative nurses play a pivotal role in the successful management of malignant hyperthermia. The fictitious case study presented in this paper describes the identification, presentation, pathophysiology, and treatment of a general anesthesia patient with fulminant malignant hyperthermia.
Topics: Anesthesia, General; Humans; Malignant Hyperthermia; Nurse's Role; Nursing Staff, Hospital; Perioperative Nursing
PubMed: 30236564
DOI: 10.1016/j.jopan.2017.04.008 -
Harefuah Jun 2020In this article we describe the treatment of a patient who developed suspicious symptoms of malignant hyperthermia syndrome during anesthesia for elective cerebral...
In this article we describe the treatment of a patient who developed suspicious symptoms of malignant hyperthermia syndrome during anesthesia for elective cerebral catheterization. We also described an up-to-date review of malignant hyperthermia, diagnosis and treatment. Details regarding the case: this is a case of a 57 year old male patient who was admitted for an elective catheterization under general anesthesia. Four hours following anesthesia induction, the patient presented with the following symptoms: a gradual increase in end tidal carbon dioxide measurements, an elevated core temperature, tachycardia, decreased oxygen saturation and excessive sweating. Arterial blood gases indicated respiratory acidosis. With a clinical diagnosis of malignant hyperthermia, the catheterization procedure was stopped. The patient was disconnected from the anesthesia machine and was ventilated with a clean ventilator with 100% oxygen. Additionally, active patient cooling was initiated along with supportive pharmacologic treatment. The patient was then moved, anesthetized and ventilated into the post anaesthesia care unit. Following a clinical and laboratory improvement the patient was extubated.
Topics: Anesthesia, General; Anesthesiology; Fever; Humans; Male; Malignant Hyperthermia; Middle Aged; Oxygen
PubMed: 32583646
DOI: No ID Found