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At least 19 recordsLinked to original sources
PubMed1999

Eltanolone (5-beta-pregnanolone) does not trigger, and attenuates halothane triggering of, malignant hyperthermia in malignant hyperthermia susceptible swine.

Eltanolone is the lipid emulsion formulation, for intravenous use, of the steroid anaesthetic 5-beta-pregnanolone. We have screened this agent in malignant hyperthermia susceptible (MHS) Landrace swine to assess its potential to trigger the Malignant Hyperthermia (MH) syndrome in MHS subjects or to influence halothane triggering of MH in such patients. Administered in anaesthetic concentration, eltanolone did not trigger the MH syndrome in MHS swine. When co-administered in low dosage with halothane, the drug prevented initiation of the MH syndrome in four of eight pigs and attenuated its progress in the remainder.

Anesthetics, Inhalation↗
PubMed2003

[Malignant hyperthermia].

Malignant hyperthermia susceptibility is a rare pharmacogenetic muscular disorder that may lead to potentially fatal complications in routine anesthesia. Malignant hyperthermia is triggered by volatile anesthetics and succinylcholine. In this article the pathophysiology of malignant hyperthermia, its signs, symptoms and treatment as well as safe anesthetic agents for individuals susceptible to malignant hyperthermia are discussed. Although progress is made in our knowledge of the genetics behind malignant hyperthermia, muscle biopsy and in-vitro contracture testing is still the only way to diagnose malignant hyperthermia susceptibility in the vast majority of cases. Danish Malignant Hyperthermia Center at the Anesthetic Department of Herlev University Hospital, DK-2730 Herlev, takes care of all testing for malignant hyperthermia in Denmark and keeps a registry of all Danes with known or suspected susceptibility to malignant hyperthermia.

Anesthetics, Inhalation↗
PubMed2002

[Malignant hyperthermia].

Malignant hyperthermia is a potentially fatal pharmacogenetic disease triggered by volatile anesthetics and/or succinylcholine. Dysregulation of intracellular calcium homeostasis is the trigger of the acute crisis. Malignant hyperthermia crisis correspond to an hypermetabolic state, which occurred acutely and interesting skeletal muscular cell. Early manifestations grouped tachycardia, tachypnea, masseter spasm, mixed acidosis and raise of the end expiratory CO2 pressure. Hyperthermia is a late sign, rhabdomyolysis is a sign of the severity of the malignant hyperthermia. The successful treatment is based on an early diagnosis, immediately interruption of triggering agents, intravenous administration of Dantrolene in sufficient dosage and starting of adequate symptomatic treatment. Prevention of this complication is based on asking the patient about genetic predisposition to malignant hyperthermia. Confirmation of the susceptibility to malignant hyperthermia can be provided by in vitro contracture test with halothane or caffeine after muscle biopsy.

Anesthesia, General↗
PubMed1997

Malignant hyperthermia.

Malignant hyperthermia is a pharmacogenetic disease of skeletal muscle characterized by hypermetabolism that occurs on exposure to a triggering agent or agents. The most common agents are halogenated inhalational anesthetics and succinylcholine, a depolarizing muscle relaxant. Patients who experience malignant hyperthermia are generally transferred to the ICU for ongoing treatment and monitoring for secondary complications of the disorder. Critical care practitioners must be both knowledgeable and competent to prevent and treat perioperative episodes of malignant hyperthermia. A thorough preoperative interview should be done to determine risk factors and susceptible patients. This article provides critical care nurses with sound information on the pathophysiology of malignant hyperthermia, the ability to assess the disease properly and treat the patient both before and after the crisis, and the ability to provide support and teaching to patients and patients' families to prevent the recurrence of malignant hyperthermia.

Animals↗
PubMed1999

[Malignant hyperthermia].

Malignant hyperthermia is a serious anesthetic complication, presenting with various manifestations, with high mortality and morbidity. There are several incomplete and abortive forms, and the clinicians must be aware of the possibility of malignant hyperthermia, and recognize the first signs of the hyperthermic crisis hyperthermia, hypermetabolism and muscular rigidity. Screening is performed by in vitro contracture testing on a muscular biopsy. Treatment is based on discontinuation of triggering agents and dantrolene administration, as well as supportive care. Cellular investigations demonstrate that the malignant hyperthermia crisis presents as an intracellular flooding with calcium, leading to an abnormal muscular contracture. Several mutations in particular involving the ryanodine receptor gene have been linked to malignant hyperthermia.

Biopsy↗
PubMed1998

Malignant hyperthermia.

Malignant hyperthermia is a rare autosomal dominant trait that predisposes affected individuals to great danger when exposed to certain anaesthetic triggering agents (such as potent volatile anaesthetics and succinylcholine). A sudden hypermetabolic reaction in skeletal muscle leading to hyperthermia and massive rhabdomyolysis can occur. The ultimate treatment is dantrolene sodium a nonspecific muscle relaxant. Certain precautions should be taken before anaesthesia of patients known to be susceptible to malignant hyperthermia. These include the prohibition of the use of triggering agents, monitoring of central body temperature and expired CO2, and immediate availability of dantrolene. In addition, careful cleansing of the anaesthesia machine of vapours of halogenated agents is recommended. If these measures are taken, the chances of an MH episode are greatly reduced. When malignant hyperthermia-does occur in the operating room, prompt recognition and treatment usually prevent a potentially fatal outcome. The most reliable test to establish susceptibility to malignant hyperthermia is currently the in vitro caffeine-halothane contracture test. It is hoped that in the future a genetic test will be available.

Anesthesia↗
PubMed1975

Malignant hyperthermia.

Malignant hyperthermia is a genetic disease that may become apparent after exposure to drugs; some of these drugs are commonly used by the oral surgeon. One in 14,000 young, apparently health individuals may be affected; mortality is 64 percent. Many cases of malignant hyperthermia can be prevented if an adequate history is obtained. Oral surgeons should be familiar with the prevention, diagnosis, and initial treatment of malignant hyperthermia.

Adolescent↗
PubMed1991

Evidence for related myopathies in exertional heat stroke and malignant hyperthermia.

Malignant hyperthermia may be a human stress syndrome, of which heat stroke is one manifestation. Two men in military service who had episodes of exertional heat stroke, and their immediate family members, were tested for susceptibility to malignant hyperthermia by in-vitro contracture tests on skeletal muscle samples. Muscle from both patients had a normal response to caffeine but an abnormal response to halothane. Muscle from the father of one patient had an abnormal response to halothane, and that from the father of the second patient had an abnormal response to ryanodine. The results indicate that clinical heat stroke may be associated with an underlying inherited abnormality of skeletal muscle that is similar, but not identical, to that of malignant hyperthermia.

Adult↗
PubMed1992

Malignant hyperthermia.

Malignant hyperthermia is a rare syndrome that occurs in genetically susceptible individuals who are exposed to frequently used inhalation anesthetics. The disorder is most common in children and young adults. It is triggered through a defect in the ability of skeletal muscles to concentrate and release calcium. Signs of malignant hyperthermia include hypercarbia, muscle rigidity and tachycardia. Temperature elevation is often a late sign of the syndrome. Treatment begins with stopping all inhaled anesthetics at the earliest sign of the syndrome. The use of dantrolene has significantly reduced mortality from malignant hyperthermia. No simple screening test exists. Family members or those with a suspicious history need to be counseled and should consider muscle biopsy and testing prior to surgery.

Adult↗
PubMed2003

Malignant hyperthermia.

Malignant Hyperthermia (MH) has been a recognized complication of general anesthesia after the first case reports in the 1940's. Since then a great deal has been discovered about the genetics, pathophysiology and treatment of this once fatal syndrome. MH is the only clinical entity specifically related to and caused by anesthetic agents. MH once triggered during anesthesia results in a profound hyper metabolic state with rise in the core temperature, increased carbon dioxide production and oxygen consumption. Death will ensue if specific treatment is not started. The incidence of fulminant MH ranges from 1:62,000 to 1: 84,000 of general anesthesia cases if succinylcholine and inhalation agents are used. Massseter muscle spasm on induction of anesthesia, with an incidence of between 1:16,000 and 1:4,000, may be a predromal indication of the development of MH. Anesthetic agents, which may trigger MH in susceptible individuals, are the depolarizing muscle relaxant, succinyl choline and all the volatile anesthetic gasses. Nitrous oxide, intravenous induction agents, benzodiazepines, opioids, and the non-depolarizing relaxants do not trigger MH. MH susceptibility is associated with certain disorders, such as Duchene muscular dystrophy, and triggering agent should not be used in these patients. Inheritance is an autosomal dominant trait with variable penetrance. The pathogenesis of MH involves the loss of control of intracellular calcium ions in skeletal muscle with resultant protracted spasm and hyper metabolism. Clinically this will progress to hypercarbia, hypoxia, hyperthermia, hyperkalemia and death will result if specific treatment is not started. Management involves immediate discontinuation of the triggering anesthetics, hyperventilation with 100% oxygen and most importantly the definitive treatment with intravenous dantrolene.The importance of instigating the use of dantrolene in cases of MH cannot be overemphasized. MH is now treatable when once it would be fatal before the availability of dantrolene. Unless of an emergent nature, surgery should be canceled following the acute phase of MH. The patient should be admitted to intensive care for at least 24 hours and dantrolene continued as recurrence has been described. It is imperative that the patient and their family are counseled, Medalert bracelets provided and registration with the Malignant Hyperthermia Association of the United States (MHAUS), encouraged. The caffeine/halothane testing of muscle biopsies is currently the most definitive test for malignant hyperthermia susceptibility. The routine use in suspected cases or the immediate family of known cases remains a matter of contention.

Anesthesia, General↗
PubMed2003

Screening for mutations in the RYR1 gene in families with malignant hyperthermia.

Malignant hyperthermia (MH) is a potentially lethal pharmacogenetic predisposition associated with a susceptibility to volatile anesthetics and depolarizing muscle relaxants that lead to a fulminant anesthetic crisis with hyperthermia, skeletal muscle rigidity, respiratory and metabolic acidosis, and muscle rhabdomyolysis. Malignant hyperthermia crises are caused by an abnormal regulation of the calcium release mechanism, which reflects the consequences of disturbed skeletal muscle calcium homeostasis. We screened 64 individuals of 27 unrelated families for the most frequently described mutations associated with MH in the genes RYR1 and CACNL1A3. We identified only one family with the Arg614Cys mutation but with a discordant segregating pattern to the in vitro contracture test (IVCT). To elucidate which other mechanism could lead to susceptibility in the members of this family, we tested it for further MH susceptibility loci. The same haplotype was shown to segregate with the individuals carrying the Arg614Cys mutation in chromosome 19; however, the other susceptible and equivocal individuals do not share this haplotype. Markers for the susceptible locus in chromosome regions 17q, 7q, 3q, and 5p did not segregate with the IVCT phenotype in the susceptible individuals, suggesting that the positivity of the IVCT could be attributable to other ambient factors.

Calcium↗
PubMed2004

Malignant hyperthermia.

Malignant hyperthermia refers to covert myopathies, which do not affect the individual during daily life activities, but may lead to life-threatening tachycardia, rigor, labile blood pressure and most importantly high-grade temperature when exposed to general anaesthesia. This conditions is mimicked by thyroid storm, neuroleptic malignant syndrome, phaeochromocytoma and sepsis. We present a presumptive case of malignant hyperthermia.

Adult↗
PubMedDate not supplied

Malignant hyperthermia.

Malignant hyperthermia (MH) is an acute syndrome that occurs in genetically susceptible persons who have been exposed to certain anesthetic agents. It can also be induced by catecholamines activated by stress. One in 200 people is at risk for developing malignant hyperthermia. The mortality rate may be as high as 15%. A thorough history obtained before surgery is of extreme importance to prevent an MH crisis and in the management of those susceptible to the disorder. If MH is suspected a diagnosis, which can be confirmed by muscle biopsy, should be ruled out before surgery.

Adult↗
PubMedDate not supplied

Malignant hyperthermia.

Malignant hyperthermia is a potentially fatal complication of general anesthesia that may occur with greater frequency in some patients with ptosis or strabismus. The history of our knowledge of this recently described condition is outlined. The clinical features, pathophysiology, and differential diagnosis are reviewed in detail. A protocol for the treatment of the acute malignant hyperthermia crisis and guidelines for the management of susceptible patients are suggested. The medicolegal implications of the occurrence of such a crisis are discussed.

Adolescent↗
PubMed1977

Malignant hyperthermia.

Malignant hyperthermia of anesthesia is a severe complication and must be treated vigorously. The anesthetic should be stopped and the core body temperature reduced. Systemic complications must be anticipated, hopefully prevented, and appropriately treated. Appropriate laboratory studies must be obtained. A comprehensive family survey may alert the physician to a tendency toward this problem. Temperature monitoring during surgery may give an early warning of malignant hyperthermia developing. I would suggest that routine temperature monitoring during surgery be considered by the anesthesia department during each general anesthetic administration.

Adult↗
PubMed1997

Detection of a novel mutation at amino acid position 614 in the ryanodine receptor in malignant hyperthermia.

Malignant hyperthermia (MH) is a potentially fatal autosomal dominant disorder of skeletal muscle and is triggered in susceptible people by all commonly used inhalation anaesthetics and depolarizing neuromuscular blocking agents. To date, eight mutations in the skeletal muscle ryanodine receptor gene (RYR1) have been identified in malignant hyperthermia susceptible (MHS) and central core disease (CCD) cases. We have screened the RYR1 gene in affected individuals for novel MHS mutations by single stranded conformational polymorphism (SSCP) analysis and have identified a G to T transition mutation which results in the replacement of a conserved arginine (Arg) at position 614 with a leucine (Leu). The Arg614Leu mutation was present in three unrelated MHS individuals of 151 investigated. The mutation was not detected in 148 normal chromosomes and segregated precisely with MHS in family members from one of the probands where DNA was available for analysis. This mutation occurs at the same position as the previously identified Arg to Cys mutation reported in all cases of porcine MH and in approximately 5% of human MH. A comparison of the phenotypes of the Arg614Leu and Arg614Cys probands is presented.

Amino Acid Sequence↗
PubMed1991

A substitution of cysteine for arginine 614 in the ryanodine receptor is potentially causative of human malignant hyperthermia.

Malignant hyperthermia (MH) is a devastating, potentially lethal response to anesthetics that occurs in genetically predisposed individuals. The skeletal muscle ryanodine receptor (RYR1) gene has been linked to porcine and human MH. Furthermore, a Cys for Arg substitution tightly linked to, and potentially causative of, porcine MH has been identified in the ryanodine receptor. Analysis of 35 human families predisposed to malignant hyperthermia has revealed the presence, and cosegregation with phenotype, of the corresponding substitution in a single family. This substitution, by analogy to the findings in pig, may be causal for predisposition to MH in this family.

Adolescent↗
PubMed2001

Malignant hyperthermia.

Malignant hyperthermia is an autosomal-dominant inherited disorder of the skeletal muscle cell characterized by a hypermetabolic response to all commonly used inhalational anaesthetics and depolarizing muscle relaxants. The clinical syndrome includes muscle rigidity, hypercapnia, tachycardia and myoglobinuria as result of increased carbon dioxide production, oxygen consumption and muscle membrane breakdown. In human beings and animals susceptible to malignant hyperthermia, it is generally accepted that an increase in the level of myoplasmic free calcium is the cause of the syndrome. Various hypotheses have been proposed to account for the increase of intracellular calcium levels, e.g. a defect in the calcium release channel of the sarcoplasmic reticulum (ryanodine receptor), an abnormality of the excitation-contraction coupling mechanisms, or alterations in second messenger systems of skeletal muscles. This review gives an overview of the main features of this disease and recent advances in research including pathophysiology, treatment, diagnosis and genetics as well as association with other disorders.

Anesthesia↗