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)

We studied the effects of succinylcholine (SCC) on serum levels of myoglobin (Mb) and CK in children under halothane or enflurane anesthesia. Forty-five children, aged 2 years 11 months to 12 years 6 months were subjected to this study. They were divided into four groups; the two groups were injected with SCC 1 mg.kg-1 intravenously to facilitate tracheal intubation and maintained with halothane (Group SH) or with enflurane (Group SE). The other two groups were intubated without the aid of SCC and maintained with halothane (Group H), or with enflurane (Group E). Mb was analyzed by an antibody radioimmunoassay technique and CK was analyzed by a modified Rosalki's method. While Mb increased in all four groups, the increase was pronounced in the SCC groups (60 min value of Group SH 2192 +/- 639 ng.ml-1, Group SE 1722 +/- 436 ng.ml-1, mean +/- SE), and the increase was significantly smaller in SCC free groups (Group H 40 +/- 12 ng.ml-1, Group E 43 +/- 9 ng.ml-1). The CK value increased only in the SCC groups to 174.1 +/- 32.8 IU.l-1 in Group SH, and to 193.6 +/- 35.7 IU.l-1 in Group SE at 60 min, respectively. These results indicate that increases in Mb and CK values were mainly induced by SCC injection and that SCC might play some roles in development of malignant hyperthermia.
Masui 1990 Sep
PMID:[Effects of succinylcholine on serum levels of myoglobin and CK in children under halothane or enflurane anesthesia]. 224 8

Malignant hyperthermia (MH) can be triggered in swine either by stress or by certain anesthetic agents. In humans, MH commonly occurs in patients previously exposed uneventfully to triggering anesthetics. This variability in expressivity of the MH syndrome is a combination of unknown genetic and environmental factors. A hypothesis was tested that a fall in rectal temperature following general anesthesia can prevent the MH syndrome in susceptible patients. Nine littermate Pietrain pigs with MH were exposed to halothane after their rectal temperatures were stabilized at 35 degrees, 36 degrees, and 37 degrees C during thiopental/nitrous oxide anesthesia. The in vivo MH metabolic, cardiopulmonary, and contracture responses were attenuated at the lower rectal temperatures. The effect of varying temperatures on biopsies of skeletal muscle from these animals showed a marked decrease in contracture response to halothane when the muscle was cooled to 25 degrees C. Studies on the Ca2+ uptake process and on Ca2+ channel-Ca2+ release properties of isolated sarcoplasmic reticulum (SR) membranes showed that increasing incubation temperatures from 25 degrees to 38 degrees C increased the Ca2+ uptake rate by the SR Ca2+ pump and also increased the probability of Ca2(+)-induced Ca2+ opening of a Ca2+ channel and the release of stored Ca2+. This study indicates that temperature can have a marked effect on the expressivity of the MH defect at the whole animal, isolated tissue, and fragmented membrane levels of organization. Since many surgical patients' temperatures decrease after induction and anesthesia, this may explain one environmental factor that determines the incidence, rate, and magnitude of the MH syndrome.
Anesthesiology 1990 Sep
PMID:Porcine malignant hyperthermia: critical temperatures for in vivo and in vitro responses. 239 29

The Authors, after a brief review of malignant hyperthermia (MH), describe the use of NLA a Harrington's surgical procedure in a malignant hyperthermia susceptible (MHS) patient. Such an anaesthesiological procedure, using benzodiazepines, droperidol, fentanyl, pancuronium and N2O/O2, didn't lead to any form of MH crisis on to complications.
Minerva Anestesiol 1989 Sep
PMID:[Use of neuroleptoanesthesia for carrying out a Harrington intervention in a patient probably susceptible to malignant hyperthermia]. 263 80

The malignant hyperthermia syndrome is widely spread in pigs of the German Landrace. This has precluded the use of halothane anesthesia. The advent of homozygous halothane-negative strains stimulated us to test whether such animals were suitable for longduration halothane anesthesia. Nine sows of the German Landrace (n = 4, homozygous halothane-positive (H+); n = 5, homozygous halothane-negative (-)) were used. Anesthesia was induced by Azaperon/Metomidat-HCL followed by intubation and exposure to halothane. All 4 halothane-positive animals died within 45-90 min following the onset of halothane administration, while all 5 halothane-negative animals survived a 4-5 h deep anesthesia without problems. Thus, homozygous hylothane-positive strains of German Landrace pigs may be surgically anesthetized for long durations with halothane anesthesia.
Dtsch Tierarztl Wochenschr 1989 Sep
PMID:[Halothane anesthesia of several hours' duration in swine of the German Landrace breed, selected for resistance to malignant hyperthermia]. 279 13

In the absence of halothane challenge, incubates of skeletal whole-muscle homogenates from malignant-hyperthermia-susceptible humans and pigs exhibit greater free fatty acid production than controls, which has been attributed to elevated phospholipase A2 activity. The present study examines lipid profiles of human muscle (vastus lateralis) prior to halothane challenge, relating the lipid profiles to the magnitude of halothane contracture response in muscle from the same biopsy. No differences in cholesterol, phospholipids or free fatty acids were observed among preparations exhibiting low, moderate or strong responses to halothane. Two fatty acids associated with triglycerides (palmitoleic and oleic) were significantly (P less than 0.05) lower in muscle demonstrating a strong response to halothane. These results do not support altered phospholipase A2 activity as a defect in malignant hyperthermia, but rather support an enhanced turnover of triglycerides in biopsied skeletal muscle from MH-susceptible humans.
Eur J Anaesthesiol 1989 Sep
PMID:Triglycerides, not phospholipids, are the source of elevated free fatty acids in muscle from patients susceptible to malignant hyperthermia. 279 95

Forty patients undergoing diagnostic muscle biopsy as part of investigation for malignant hyperthermia (MH) were given atracurium 0.45 +/- 0.10 mg kg-1 for muscle paralysis. The neuromuscular blockade was antagonized with neostigmine 2.4 +/- 0.4 mg given with glycopyrrolate 0.47 +/- 0.09 mg. Rectal, muscle and skin temperatures and blood lactate concentration and venous Pco2 were measured before, during and after anaesthesia. Susceptibility to MH was established by in vitro contracture tests according to the regimen of the European MH Group. Fifteen patients were susceptible to MH (MHS), 19 were MH-negative (MHN) and six were MH-equivocal (MHE). No side effects of the drugs were noted. There were no differences between the three groups of patients in any of the measurements.
Br J Anaesth 1986 Sep
PMID:Atracurium and its antagonism by neostigmine (plus glycopyrrolate) in patients susceptible to malignant hyperthermia. 309 48

Malignant hyperthermia is a potentially lethal syndrome that can be triggered by inhaled anesthetics. Thus, it may be appropriate to utilize equipment that minimizes exposure of susceptible patients to inhaled anesthetics. The rate of release of anesthetic stored in anesthesia delivery systems is unknown. To determine residual anesthetic concentrations, the washout rates of halothane and isoflurane were compared, and the effects of a 1-l/min and a 10-l/min fresh gas flow were evaluated. Halothane concentrations were also measured in samples taken from the fresh gas outlet and the Y-piece of the circle system during four separate studies in which various components of the anesthesia system were replaced. In each study an Ohio Modulus anesthesia machine equipped with an Air-Shields ventilator was exposed to 2% halothane for 18 h. Anesthetic concentrations were determined by a gas chromatograph having a sensitivity of 0.1 ppm. Isoflurane washed out 3-4 times faster than halothane. Residual halothane concentration was approximately equal to tenfold greater when the fresh gas flow was 1 l/min rather than 10 l/min: 194 versus 19 ppm after 1 h of washout. Using a 10-l/min fresh gas flow, halothane concentrations in samples obtained from the Y-piece were similar with original or fresh soda lime but were more than tenfold lower after the fresh gas outlet hose and circle system were replaced (approximately equal to 50 ppm vs. approximately equal to 5 ppm after 5 min of washout).(ABSTRACT TRUNCATED AT 250 WORDS)
Anesthesiology 1988 Sep
PMID:Preparation of anesthesia machines for patients susceptible to malignant hyperthermia. 316 44

The electrophysiological effects of an intravenous dantrolene infusion (10 mg kg-1) were evaluated in healthy, anaesthetized dogs by intracardiac electrophysiological study. Dantrolene administration resulted in a significant prolongation of the refractory periods of the right atrium and ventricle, while the functional refractory period of the AV node was not altered. A slight increase of AV nodal conduction, measured as atrial-His bundle interval, without any change in infranodal conduction, measured as His bundle-ventricular interval, was observed during sinus rhythm. Dantrolene had no significant effects on surface ECG parameters. We conclude that intravenously administered dantrolene, at the maximal recommended doses, has primary effects on electrophysiological parameters. These findings support the hypothesis that the beneficial effects of dantrolene on cardiac arrhythmias associated with malignant hyperthermia may be related to its intrinsic activity on the electrophysiological properties of the heart, but confirmation requires further investigations on induced arrhythmias in experimental models.
Eur J Anaesthesiol 1987 Sep
PMID:Electrophysiological effects of intravenous dantrolene on canine heart. 342 81

Findings in 13 patients with central core disease are reported and compared with 62 patients from the literature. Patients with central core disease show wide variation in the clinical spectrum of muscle involvement. Some will have no symptoms and be completely normal on examination, many will show mild to moderate weakness, and a small number will be severely affected. Muscle atrophy is a frequent finding, but progression of muscle weakness is rare and occurs only slowly. Musculoskeletal deformities, including kyphoscoliosis, congenital hip dislocation, feet deformities, and joint contractures, are often seen but generally do not alter the natural history of the disease. Surgical treatment may be required for some of these abnormalities. No relationship appears to exist between the degree of muscle weakness and the presence or type of musculoskeletal deformities. Cardiac abnormalities have rarely been reported in association with central core disease. Mitral valve prolapse was noted in 3 of our patients and 2 others had cardiac arrhythmias. Central core disease and susceptibility to malignant hyperthermia was present in all 11 patients we studied (2 patients with central core disease did not have studies for malignant hyperthermia). These 11 patients came from 4 families. All patients with central core disease should be considered at risk for malignant hyperthermia unless in-vitro contracture tests show that the particular patient is free of the trait.
Medicine (Baltimore) 1987 Sep
PMID:Central core disease. Clinical features in 13 patients. 362 47

It has been speculated that, in malignant hyperthermia-susceptible (MHS) individuals, an abnormality of sympathetic activity is seen during stressful situations, such as exercise. The authors investigated whether muscle metabolism in eight MHS subjects, at rest and during moderate and heavy short-term exercise, is different then that in normals. Leg exchange of energy substrates (glucose, lactate, and glycerol) was quantified by measuring leg blood flow and arterial-venous concentration differences. Muscle biopsies were also performed, and ATP, glycogen, and lactate were analyzed. Catecholamines and oxygen uptake were also measured. The study was performed at rest with subjects in the supine position and during two periods (40% and 80% of the subjects maximal oxygen uptake, respectively) on a bicycle ergometer. The principal finding of the study was that there was no major difference in oxygen uptake or leg exchange of glucose, lactate, and glycerol between MHS-subjects and previously standard normals during different grades of exercise. Furthermore, muscle metabolites and plasma catecholamines did not differ between the groups. This study indicates a normal sympathetic activity and muscle metabolism in MHS subjects during rest, as well as during moderate and severe exercise. The authors' results do not support the opinion that persons with positive in vitro tests for MH should restrict their physical activity.
Anesthesiology 1987 Sep
PMID:Effects of graded exercise on leg exchange of energy substrates in malignant hyperthermia susceptible subjects. 363 4


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