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Advances in Anesthesia Dec 2019
Review
Topics: Calcium Channels, L-Type; Calcium-Binding Proteins; Calsequestrin; Comorbidity; Dantrolene; Genetic Predisposition to Disease; Humans; Incidence; Malignant Hyperthermia; Mitochondrial Proteins; Muscle Relaxants, Central; Perioperative Care; Prevalence; Ryanodine Receptor Calcium Release Channel
PubMed: 31677658
DOI: 10.1016/j.aan.2019.08.003 -
Cureus May 2021Malignant hyperthermia (MH) is a genetic skeletal muscle disorder characterized by hypermetabolic crisis usually triggered by anesthetic drugs. Non-anesthesia-triggered...
Malignant hyperthermia (MH) is a genetic skeletal muscle disorder characterized by hypermetabolic crisis usually triggered by anesthetic drugs. Non-anesthesia-triggered or awake MH is rare or under-reported. Other than anesthetic drugs, identified common triggers are exercise, fever, and viral infection. The literature does not report any awake MH cases triggered by dengue fever. We report a possible case of dengue fever-triggered awake MH. The main aim of reporting this case is to raise awareness of possible malignant hyperthermia in dengue patients and of a clinical grading system (CGS) for early diagnosis, as early treatment with dantrolene sodium reduces the mortality rate.
PubMed: 34159023
DOI: 10.7759/cureus.15121 -
British Journal of Anaesthesia Dec 2020
Topics: Electronic Health Records; Genetic Testing; Genomics; Mutation
PubMed: 32958205
DOI: 10.1016/j.bja.2020.08.046 -
The American Journal of Emergency... Nov 2022Serotonin syndrome is a rare, frequently misdiagnosed, serious condition with high morbidity. (Review)
Review
INTRODUCTION
Serotonin syndrome is a rare, frequently misdiagnosed, serious condition with high morbidity.
OBJECTIVE
This review highlights the pearls and pitfalls of serotonin syndrome, including diagnosis, initial resuscitation, and management in the emergency department (ED) based on current evidence.
DISCUSSION
Serotonin syndrome is a potentially deadly toxidrome marked by excess serotonin receptor activity or neurotransmission. Features of serotonin syndrome include 1) neuromuscular excitation such as tremor, hyperreflexia, and clonus; 2) autonomic dysfunction such as tachycardia, hypertension/hypotension, and hyperthermia; and 3) altered mental status such as agitation, delirium, and coma. Although serotonin syndrome may be more obvious in patients who have overdosed on serotonergic agents such as serotonin reuptake inhibitors (SSRIs), multiple other medications may also cause serotonin syndrome. Alternative diagnoses such as sepsis, neuroleptic malignant syndrome, and decompensated hyperthyroidism should be considered. The primary components of therapy include stopping the offending agent and supportive care, which focuses on agitation control, monitoring for and treating hyperthermia, and managing autonomic instability.
CONCLUSIONS
An understanding of serotonin syndrome can assist emergency clinicians in diagnosing and managing this disease.
Topics: Humans; Serotonin Syndrome; Selective Serotonin Reuptake Inhibitors; Prevalence; Neuroleptic Malignant Syndrome; Receptors, Serotonin
PubMed: 36057215
DOI: 10.1016/j.ajem.2022.08.030 -
Notfall & Rettungsmedizin 2021These guidelines of the European Resuscitation Council (ERC) Cardiac Arrest under Special Circumstances are based on the 2020 International Consensus on Cardiopulmonary... (Review)
Review
These guidelines of the European Resuscitation Council (ERC) Cardiac Arrest under Special Circumstances are based on the 2020 International Consensus on Cardiopulmonary Resuscitation Science with Treatment Recommendations. This section provides guidelines on the modifications required for basic and advanced life support for the prevention and treatment of cardiac arrest under special circumstances; in particular, specific causes (hypoxia, trauma, anaphylaxis, sepsis, hypo-/hyperkalaemia and other electrolyte disorders, hypothermia, avalanche, hyperthermia and malignant hyperthermia, pulmonary embolism, coronary thrombosis, cardiac tamponade, tension pneumothorax, toxic agents), specific settings (operating room, cardiac surgery, cardiac catheterization laboratory, dialysis unit, dental clinics, transportation [in-flight, cruise ships], sport, drowning, mass casualty incidents), and specific patient groups (asthma and chronic obstructive pulmonary disease, neurological disease, morbid obesity, pregnancy).
PubMed: 34127910
DOI: 10.1007/s10049-021-00891-z -
Minerva Anestesiologica Aug 2021Children with symptoms of hypotonia (reduction of postural tone of lower limbs and trunk with or without changes in phasic tone) are frequently anesthetized for... (Review)
Review
Children with symptoms of hypotonia (reduction of postural tone of lower limbs and trunk with or without changes in phasic tone) are frequently anesthetized for diagnostic and therapeutic interventions. This review outlines the underlying causes and classifications, and the anesthesiologic pre- and peri-operative management of hypotonic children. Hypotonia may have a large range of etiologies that are categorized into central and peripheral hypotonia. A multidisciplinary approach towards the (differential) diagnosis of the underlying cause of the symptoms in cooperation with a pediatrician and/or pediatric neurologist is emphasized. Anesthetic management involves the anticipation of an increased risk in difficult airway management because of macroglossia, reduced mouth opening, obesity and limited neck mobility, which increases with age. There are no specific restrictions towards the use of intravenous or inhalational anesthetics. Short acting opioids and hypnotics, avoiding neuromuscular blockade, and locoregional techniques are preferred. Most patients are sensitive to the cardiac and depressive effects of anesthetics and all dystrophic myopathies are considered at risk of malignant hyperthermia. Depolarizing neuromuscular blockers are contraindicated. The use of a peripheral nerve stimulator is recommended to detect the severity of muscle relaxation before extubating. Accurate control and management of IV fluids, electrolytes and temperature is mandatory. Adequate postoperative pain treatment is essential to limit stress and metabolic alteration. Preferably a locoregional technique is used to reduce the increased risk of respiratory depression. A multidisciplinary preoperative approach taking into account the differential diagnosis of the underlying disease of the floppy child is recommended.
Topics: Anesthetics, Inhalation; Child; Electric Stimulation Therapy; Humans; Muscle Hypotonia; Obesity; Pain, Postoperative
PubMed: 33432795
DOI: 10.23736/S0375-9393.20.15011-9 -
A&A Practice Jun 2021
Topics: Hemodynamics; Humans; Hyperthermia; Malignant Hyperthermia
PubMed: 34161308
DOI: 10.1213/XAA.0000000000001472 -
Journal of Perianesthesia Nursing :... Aug 2022Malignant hypothermia (MH) is a potentially fatal hypermetabolic reaction of skeletal muscle. It is an autosomal dominant disorder that generally occurs in people with...
Malignant hypothermia (MH) is a potentially fatal hypermetabolic reaction of skeletal muscle. It is an autosomal dominant disorder that generally occurs in people with RYR1, CACNA1S, or STAC3 mutations. And these genetic abnormalities often cause the imperfection of calcium release channels of skeletal muscle. The incidence of MH among different racial groups across the world ranges from approximately 1:5,000-1:250,000, but there is no national statistic MH incidence in China. It is not clear whether there are racial or regional differences in the incidence, but patients under 18 years old may be more affected. MH can be triggered by anesthetics, or other stimuli, such as strenuous exercise, heat-stroke, and emotional stress. While viral infection, statins, hyperglycemia, and muscle metabolic dysfunctions might accelerate the onset of MH. The onset of MH is insidious and rapid, with the preclinical stage characterized by rigidity of the masseter muscle, a high level of end-tidal carbon dioxide, and a sharp and persistent increase in body temperature. Medical history, family history, clinical presentation, in vitro caffeine-halothane contracture testing (IVCT/CHCT) and genetic testing are commonly diagnostic methods of MH. As soon as the onset of MH is suspected, immediate cessation of exposure to stimuli, call for professional support, and access to dantrolene are the highest priorities. For symptomatic treatment, "5C principles" were summarized as an algorithm to guide clinicians.
Topics: Adolescent; Caffeine; China; Halothane; Humans; Malignant Hyperthermia; Mutation
PubMed: 35414440
DOI: 10.1016/j.jopan.2021.08.018 -
Skeletal Muscle Nov 2020The RYR1 gene, which encodes the sarcoplasmic reticulum calcium release channel or type 1 ryanodine receptor (RyR1) of skeletal muscle, was sequenced in 1988 and RYR1... (Review)
Review
The RYR1 gene, which encodes the sarcoplasmic reticulum calcium release channel or type 1 ryanodine receptor (RyR1) of skeletal muscle, was sequenced in 1988 and RYR1 variations that impair calcium homeostasis and increase susceptibility to malignant hyperthermia were first identified in 1991. Since then, RYR1-related myopathies (RYR1-RM) have been described as rare, histopathologically and clinically heterogeneous, and slowly progressive neuromuscular disorders. RYR1 variants can lead to dysfunctional RyR1-mediated calcium release, malignant hyperthermia susceptibility, elevated oxidative stress, deleterious post-translational modifications, and decreased RyR1 expression. RYR1-RM-affected individuals can present with delayed motor milestones, contractures, scoliosis, ophthalmoplegia, and respiratory insufficiency.Historically, RYR1-RM-affected individuals were diagnosed based on morphologic features observed in muscle biopsies including central cores, cores and rods, central nuclei, fiber type disproportion, and multi-minicores. However, these histopathologic features are not always specific to RYR1-RM and often change over time. As additional phenotypes were associated with RYR1 variations (including King-Denborough syndrome, exercise-induced rhabdomyolysis, lethal multiple pterygium syndrome, adult-onset distal myopathy, atypical periodic paralysis with or without myalgia, mild calf-predominant myopathy, and dusty core disease) the overlap among diagnostic categories is ever increasing. With the continuing emergence of new clinical subtypes along the RYR1 disease spectrum and reports of adult-onset phenotypes, nuanced nomenclatures have been reported (RYR1- [related, related congenital, congenital] myopathies). In this narrative review, we provide historical highlights of RYR1 research, accounts of the main diagnostic disease subtypes and propose RYR1-related disorders (RYR1-RD) as a unified nomenclature to describe this complex and evolving disease spectrum.
Topics: Animals; Humans; Neuromuscular Diseases; Phenotype; Ryanodine Receptor Calcium Release Channel; Terminology as Topic
PubMed: 33190635
DOI: 10.1186/s13395-020-00243-4 -
Advanced Emergency Nursing Journal
PubMed: 33915558
DOI: 10.1097/TME.0000000000000354