Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0024591 (malignant hyperthermia)
 2,353 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The authors used dantrolene as a prophylactic during ECT with a depressed patient susceptible to malignant hyperthermia. Succinylcholine and tricyclic and MAO-inhibiting antidepressants can precipitate malignant hyperthermia and were thus avoided.
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PMID:ECT use for a patient with malignant hyperthermia. 709 36

We report on the occurrence of cardiac arrests within a few minutes following succinylcholine in 9 children, all of whom were later shown to have occult neuromuscular disease. Five of the children did not survive the catastrophic event. The anaesthetist in most cases, when discussing premedication, got the impression that the patients were in good health; just in 2 children were there indications of myopathy. Myopathic children coming to surgery and anaesthesia are rare. In these cases the administration of succinylcholine is contraindicated. But the anaesthetist must be aware of the fact that a small number of paediatric patients with unknown/subclinical myopathies might be referred to him. In these cases, without warning muscle rigor, bradycardia and hyperkalemia cardiac arrest may develop within minutes following administration of succinylcholine. The anaesthetist must be prepared for such a challenging event--particularly mentally. Misinterpretation of the symptoms as signs of malignant hyperthermia should be excluded. Resuscitation must start without delay and must continue for more than 30 minutes. Therapeutic attempts to lower extracellular potassium with glucose and insulin must fail for pharmacokinetic reasons. Therapy with intravenous calcium under control of the e.c.g. seems to be the only rational approach to the problem. It is suggested that in every healthy child coming to anaesthesia the physician should consider whether relaxation could not be achieved by other agents. Succinylcholine may well be defined as a "membrane poison"--especially considering the efflux of potassium, myoglobin and creatine kinase from the intracellular space into the bloodstream. The answer to the question asked in the title must therefore be: definitely--yes.
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PMID:[Should the use of succinylcholine in pediatric anesthesia be re-evaluated?]. 836 12

The frequency of malignant hyperthermia in the Netherlands is about 1 in 200,000 anaesthesias. Five times a year, an anaesthetic procedure will be complicated by a malignant hyperthermic metabolic disturbance, which can cause death if treatment is not instituted rapidly, by the administration of dantrolene. Suxamethonium and all the anaesthetic vapours can trigger such a reaction. Malignant hyperthermia patients are healthy patients who have a mutation of the ryanodine receptor gene RYR. Predisposition to malignant hyperthermia is inherited as an autosomal dominant condition. So far a genetic malignant hyperthermia test is not available because of genetic heterogeneity. The in-vitro contracture test in skeletal muscle is currently used as a diagnostic test for malignant hyperthermia. Patients who are likely to be at risk based on a clinical grading score, and family members with at least a 25% chance of inheriting malignant hyperthermia, are eligible for this test.
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PMID:[Malignant hyperthermia as a complication of anesthesia: predisposition is hereditary]. 919 May 36

To investigate a possible link of malignant hyperthermia to capture myopathy, between June 1990 and July 1993 we anesthetized four black-tailed deer (Odocoileus hemionus columbianus) and challenged them with halothane and succinylcholine. Halothane had no significant effect on oxygen consumption. Succinylcholine significantly (P < 0.05) increased cardiac output (mean +/- SD), from 2.94 +/- 1.05 l/min to 5.26 +/- 1.79 l/min, and oxygen consumption, from 5.5 +/- 2.1 ml/kg/min to 10.1 +/- 2.9 ml/kg/min. Muscle biopsy specimens tested for malignant hyperthermia susceptibility responded normally to halothane and caffeine. We conclude that these deer did not experience malignant hyperthermia; suggesting no link to capture myopathy.
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PMID:Evaluation for malignant hyperthermia susceptibility in black-tailed deer. 935 70

The clinical pharmacology of neuromuscular blocking agents is described. During neuromuscular blockade, succinylcholine attaches to receptors in the motor end plate and depolarizes the neuromuscular junction, making the end plate refractory to acetylcholine. The nondepolarizing relaxants have a structure similar to that of succinylcholine and bind to the same receptors. Instead of depolarizing the junction, they block acetylcholine from binding to the receptor and cause channel blockade. As the concentration of nondepolarizing relaxant increases relative to acetylcholine, neuromuscular transmission is compromised. This relationship is used clinically to facilitate recovery from nondepolarizing agents. Succinylcholine is popular because its onset is faster than that of the nondepolarizing relaxants and metabolism by pseudocholinesterase clears it quickly. It is commonly given as an i.v. bolus to facilitate tracheal intubation. The onset of these agents varies widely and is dose dependent. Large doses are usually given to hasten the onset of paralysis; subsequent doses are adjusted according to response. The nondepolarizing agents interact with inhaled anesthetics, magnesium, and many antimicrobials. Drugs like neostigmine, edrophonium, and pyridostigmine antagonize neuromuscular blockade; an anticholinergic drug is typically administered to counteract the cardiovascular effects. The most serious adverse effects of succinylcholine are malignant hyperthermia syndrome, masseter muscle rigidity, and bradycardia. Some nondepolarizing relaxants (atracurium, mivacurium, and pancuronium) are associated with histamine release, occasionally causing serious hypotension and tachycardia. Neuromuscular blocking agents are essential to anesthesia. Older compounds produce greater toxicity than newer compounds, and several of these older compounds therefore are no longer in clinical use.
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PMID:Clinical pharmacology of neuromuscular blocking agents. 1043 10

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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PMID:No response to trigger agents in a malignant hyperthermia-susceptible patient. 1217 9

Familial polymorphic (catecholaminergic) ventricular tachycardia is an arrhythmogenic cardiac disorder caused by mutations of the myocardial isoform of the ryanodine receptor gene (RyR2). Mutations of the corresponding gene in the skeletal muscle (RyR1) predispose its carriers to malignant hyperthermia upon use of volatile anesthetics or succinylcholine, which further deteriorate the inherited intracellular calcium release disorder. We report a series of patients with cardiac RyR defects who underwent general anesthesia without complications. Succinylcholine and volatile anesthetics did not have a clinically significant effect on RyR2 defects.
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PMID:Volatile anesthetics and succinylcholine in cardiac ryanodine receptor defects. 1527 19

A 3-year-old Thai boy underwent open reduction and internal fixation with K-wire of condylar fracture of humerus under general anesthesia. The patients developed generalized muscle regidity, masseter muscle spasm, elevated creatinine kinase, high temperature (39.3 C), inappropriate tachycardia, and arterial base excess was more than-8 mEq/L. The clinical grading scale of diagnosis of malignant hyperthermia was 58 (grade D6; almost certain malignant hyperthermia). Succinylcholine has been identified as the trigger agent, as other possible trigger agents were not involved. The treatment included hyperventilation, external cooling and cold IV fluids without administration of dantrolene. The patient fully recovered and discharged on day 12. This case report showed an incidence of malignant hyperthermia of approximated 1:150,000 in Thai Anesthesia Incidents Study (THAI Study).
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PMID:Malignant hyperthermia: a case report in Thai Anesthesia Incidents Study (THAI Study). 1686 89

Suxamethonium is the only depolarising neuromuscular blocking agent, which is still being widely used during general anaesthesia. Some of its unique properties rank suxamethonium as an ideal neuromuscular blocking agent i.e. the fast onset of muscle paralysis and spontaneous neuromuscular block reversal. However, the agent may trigger malignant hyperthermia, hyperkaliaemia, severe bradycardia and other complications, which have to be considered. Due to differences in postsynaptic nicotine receptor structure and functional insufficiency of the neuromuscular junction, paediatric patients when compared to adults, are more sensitive to potential side effects when suxamethonium is administered. Malignant hyperthermia is an important risk factor. Ryanidine receptors located in the sarcoplasmic/endoplasmic reticulum membrane are responsible for the release of Ca2+ from intracellular stores and trigger this complication.The risk of hyprethermia increases in children when some neurologic and muscle diseases coexist. Nowadays, in rapid sequence induction of anaesthesia, suxamethonium may be replaced with rocuronium - a non-depolarising muscle relaxant which provides the intubating conditions similar to suxamethonium. The rocuronium-induced neuromuscular blockade, which lasts longer than blockade following suxamethonium, is reversed with sugammadex - a new selective relaxant binding agent. Despite new agents and methods, suxamethonium still remains the drug of choice for muscle relaxation for intubation in children.
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PMID:[Is suxamethonium still useful for paediatric anaesthesia?]. 2201 24

Succinylcholine has been indicted on account of the diverse risks associated with its administration, which include hyperkalemia, vagal arrest, and malignant hyperthermia. However, it provides excellent intubating conditions reliably and quickly, characteristics that sustain its appeal for use in the intensive care unit, where airway management is challenging. There is an increasing body of evidence, outlined here, that rocuronium is an acceptable alternative to succinylcholine.
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PMID:Rapid sequence induction with rocuronium - a challenge to the gold standard. 2184 80


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