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Query: UMLS:C0024591 (malignant hyperthermia)
 2,353 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We have used the Leiden anaesthesia simulator, which makes use of a standard anaesthesia machine and monitors, and realistically simulates the anaesthesia work place. After obtaining informed consent, 28 anaesthetists and anaesthesia trainees in one hospital took part in the study. All participants were exposed to a pre-scripted simulated "control" scenario of anaphylactic shock (phase 1). The sessions were videotaped and the performances of individual participants were evaluated using a standardized scoring scheme. During phase 2, the participants were allocated randomly to undergo training in the management of either anaphylactic shock (group A, n = 13) or malignant hyperthermia (group B, n = 15) on the simulator. After 4 months, each participant underwent a blinded evaluation session with a pre-scripted "test" scenario of malignant hyperthermia (phase 3). These sessions were also videotaped and evaluated as for phase 1. The participants in group B responded more quickly, treated better and deviated less from the accepted procedure during phase 3 than those in group A. The total performance of participants in group B during phase 3 was significantly better than those in group A. We conclude that training on an anaesthesia simulator does improve the performance of anaesthetists in dealing with emergencies during anaesthesia.
Br J Anaesth 1994 Sep
PMID:Does training on an anaesthesia simulator lead to improvement in performance? 794 51

Neuroleptic malignant syndrome (NMS) and malignant hyperthermia (MH) may have a common pathogenic mechanism; therefore, it has been suggested that known triggering agents for MH (such as succinylcholine) should be avoided in patients with NMS. Electroconvulsive therapy (ECT) continues to play a major therapeutic role in contemporary psychiatry, and succinylcholine has been the muscle relaxant of choice in attenuating violent muscle contractions induced by ECT. Mivacurium is a non-depolarizing muscle relaxant with a relatively rapid onset and a short duration of action, and to date it has been proved safe in MH-susceptible patients. In this case report, following succinylcholine use during ECT, a patient with NMS developed an increase in temperature and serum creatine kinase (CK) level, possibly due to an MH reaction. Since the patient's mental status necessitated further ECT, mivacurium was administered during subsequent treatment and resulted in effective attenuation of muscle contractions without elevation of patient temperature or CK levels. In addition, there was no marked prolongation of the anaesthetic. Mivacurium is a suitable agent for patients with NMS undergoing ECT, as it has not been associated with precipitation of an MH response.
Can J Anaesth 1994 Sep
PMID:Neuroleptic malignant syndrome and mivacurium: a safe alternative to succinylcholine? 795 1

Central core disease (CCD) is a morphologically distinct, autosomal dominant myopathy with variable clinical features. A close association with malignant hyperthermia (MH) has been identified. Since MH and CCD genes have been linked to the skeletal muscle ryanodine receptor (RYR1) gene, cDNA sequence analysis was used to search for a causal RYR1 mutation in a CCD individual. The only amino acid substitution found was an Arg2434His mutation, resulting from the substitution of A for G7301. This mutation was linked to CCD with a lod score of 4.8 at a recombinant fraction of 0.0 in 16 informative meioses in a 130 member family, suggesting a causal relationship to CCD.
Nat Genet 1993 Sep
PMID:A mutation in the human ryanodine receptor gene associated with central core disease. 822 Apr 22

Central core disease (CCD) of muscle is an inherited myopathy which is closely associated with malignant hyperthermia (MH) in humans. CCD has recently been shown to be tightly linked to the ryanodine receptor gene (RYR1) and mutations in this gene are known to be present in MH. Mutation screening of RYR1 has led to the identification of two previously undescribed mutations in different CCD pedigrees. One of these mutations was also detected in an unrelated MH pedigree whose members are asymptomatic of CCD. The data suggest a model to explain how a single mutation may result in two apparently distinct clinical phenotypes.
Nat Genet 1993 Sep
PMID:Mutations in the ryanodine receptor gene in central core disease and malignant hyperthermia. 822 Apr 23

This article provides an overview of pediatric post anesthesia care. It highlights important aspects of care that are frequently encountered in practice or have the potential for being problematic. These include airway management, fluid maintenance, the treatment of seizures, thermoregulation, the management of malignant hyperthermia, the identification and treatment of emergence delirium, and the availability of appropriate emergency equipment and medications.
Nurs Clin North Am 1993 Sep
PMID:The pediatric patient in the post anesthesia care unit. 836 23

We have examined the phenotypic expression of several parameters associated with malignant hyperthermia (MH) susceptibility in three groups (homozygous normal, homozygous abnormal and heterozygous) of Yorkshire/Duroc swine genotyped by a mutation in the ryanodine receptor. Subgroups of homozygous abnormals were classified further by the appearance or absence of muscle rigidity on prolonged in vivo challenge with halothane and suxamethonium. Four swine heterozygous for the proposed MH mutation were indistinguishable from five homozygous normal swine in temperature, heart rate, lactate concentrations, base excess and pH determined during the prolonged halothane and suxamethonium challenge. Resting creatine kinase concentrations, the in vivo barnyard challenge, the in vitro contracture response of skeletal muscle to 3% halothane and the threshold for Ca(2+)-induced Ca2+ release were also similar for subgroups of homozygous normals and heterozygotes. Therefore, inheritance of only one allele carrying the defect in the ryanodine receptor does not significantly alter phenotypes associated with MH susceptibility in this strain of swine. As four swine homozygous for the proposed MH defect did not exhibit rigidity and three of these had no other signs of MH on prolonged halothane and suxamethonium challenge, we conclude that the reported mutation in the ryanodine receptor may be necessary, but is not sufficient, for consistently eliciting the malignant hyperthermia syndrome. These findings suggest that a modulator of the syndrome may explain variability within individuals in human MH.
Br J Anaesth 1993 Sep
PMID:Phenotypes associated with malignant hyperthermia susceptibility in swine genotyped as homozygous or heterozygous for the ryanodine receptor mutation. 839 25

Malignant hyperthermia (MH) is a rare myopathy inducing severe accident when carriers are exposed to triggering agents. MH susceptibility (MHS) is assessed by pharmacological tests performed on muscle strips. Exertional heat stroke (EHS) is a severe accident occurring during long and strenuous exercise. It has been observed that numerous EHS patients are susceptible to MH according to pharmacological tests. Because most of those EHS-MHS subjects were soldiers, we hypothesize that military duty could select subjects with infraclinical myopathy and therefore would increase the MHS:EHS subject ratio.
Med Hypotheses 1995 Sep
PMID:On the nature of the link between malignant hyperthermia and exertional heatstroke. 856 50

1. Sudden Death Syndrome (SDS) is a disease of well-developed, predominantly male broiler chickens where death appears to occur because of cardiovascular failure. The role of skeletal muscle sarcoplasmic calcium regulation as a potential cause of SDS has been investigated. 2. Calcium regulation of skeletal muscle sarcoplasmic reticulum was compared between broiler and Leghorn chickens. Calcium regulation matured from the 2nd to the 11th week and, at any age, broiler chickens showed significantly lower calcium transport rates and transport efficiencies. The mechanism of calcium transport in broiler chickens was more energy-consuming than that of the Leghorn chickens. 3. Sarcoplasmic calcium regulation is pivotal for muscle metabolism. As in porcine malignant hyperthermia, weaker calcium regulation might lead to hyperactivation of skeletal muscle, followed by elevated lactic acid concentrations and cardiovascular failure.
Br Poult Sci 1995 Sep
PMID:Skeletal muscle sarcoplasmic calcium regulation and sudden death syndrome in chickens. 859 98

We present the case of a patient affected with Williams syndrome (WS), who developed a suspected malignant hyperthermia (MH) reaction to general anesthesia. The proximity to the WS region of the gene encoding the L-type voltage-gated calcium channel alpha 2/delta-subunit (CACNL2A) on 7q11.23-q21.1, previously shown to be closely linked to some forms of MH susceptibility, prompted us to investigate whether this gene is deleted in WS. Linkage studies and fluorescence in situ hybridization analysis demonstrated that the CACNL2A locus is localized outside the WS deleted region.
Hum Genet 1996 Sep
PMID:Anesthesiologic problems in Williams syndrome: the CACNL2A locus is not involved. 870 1

First described in 1960, malignant hyperthermia (MH) is a relatively rare disorder of muscle metabolism triggered by specific anesthetic agents and presenting as a rapidly evolving intraoperative crisis. The syndrome is more prevalent among children (1/15,000 exposures to anesthesia) than adults (1/50,000 exposures), but has not been thought to occur in children younger than 1 year of age. This is a case report of a 3-month-old, 4.85 kg white male infant who developed MH while undergoing repair of a right inguinal hernia. The case is unique not only with respect to the age of the patient but also with the extremely elevated creatine phosphokinase (CPK) 13 hours postoperatively.
J Med Assoc Ga 1996 Sep
PMID:Malignant hyperthermia in a 3-month-old infant: a case report. 887 Apr 51


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