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)

Thirteen of 31 Belgian Landrace pigs developed malignant hyperthermia (MH) after breathing halothane. A short period of exercise 1 h before the administration of the triggering agent increased the incidence of the syndrome to 100% in eight similar pigs. Clinical symptoms were more marked and developed more rapidly in the exercised pigs. All the reacting pigs became typically acidotic, developed rigor and died. Serum Na+, K+, Ca2+, c.p.k., l.d.h. and protein concentrations were increased to a variable extent during the reaction and there was an increase in p.c.v. also. No hyperglycaemia was detected in pigs which were rested before receiving halothane. Four of the eight exercised pigs became markedly hyperglycaemic and plasma noradrenaline increased to higher values. Phosphocreatine and ATP decreased to low values and lactate increased in the muscles of all pigs which reacted. At the time of death, muscle glycogen had decreased significantly in the rested, but not in the exercised, MH pigs.
Br J Anaesth 1976 Sep
PMID:Malignant hyperthermia in Belgian Landrace pigs rested or exercised before exposure to halothane. 97 90

Data are presented on ten cases of anaesthesia-induced malignant hyperthermia in Norway. Seven of the patients died, three recovered. The fatal cases were all boys in the age group 11-20 years. This age and sex distribution suggests that puberty with the increase in androgens is a precipitating factor in malignant hyperthermia. One of the victims who survived was a 4 1/2-year-old pseudohermaphrodite girl with the adrenogenital syndrome. The coincidence of malignant hyperthermia in a patient with such a rare syndrome points to the excessive formation of androgens in patients with this syndrome as a predisposing factor. The indications for surgery were traumatic injuries in five cases, congenital abnormalities in three and appendicitis in two cases. These conditions in themselves may cause an increased sensitivity to suxamethonium. One patient received only hexobarbitone, halothane and suxamethonium. After the last drug jaw rigidity and temperature rise to 41.3 degrees C prompted the anaesthetist to end the anaesthetic. The fact that the patient survived proves that suxamethonium induced jaw rigidity is valuable as a warning. The absence of cardiovascular depression after procaine 3.5 g in one patient is ascribed to the correction of acidosis at the time of infusion of this drug. It is suggested that procaine should be withheld until other measures such as cooling, correction of acidosis and steroid therapy have been tried.
Can Anaesth Soc J 1976 Sep
PMID:Ten cases of malignant hyperthermia in Norway. 97 60

A 32-year-old man survived malignant hyperthermia which developed in conjunction with laparotomy. Muscles biopsy indicated acute necrosis of muscle fibers as well as changes consistent with a latent myopathy. Clinical, laboratory and morphological studies of the relatives revealed familial subclinical myopathy. The hyperthermia resulting from the anaesthesia is thought to be casually related to the myopathy, and members of the family have been warned against this potential risk.
Dtsch Med Wochenschr 1975 Sep 26
PMID:[Clinical and morphological findings in a case of malignant hyperthermia (author's transl)]. 115 6

The relatives of a patient who died from malignant hyperpyrexia were examined clinically and biochemically. One fo the deceased's children, an identical twin, undergoing an anesthetic, was found to develop malignant hyperpyrexia on exposure to nitrous oxide. The creatine phosphokinase concentrations in this family were unhelpful as a method of identifying susceptible individuals.
Br J Anaesth 1975 Sep
PMID:Malignant hyperpyrexia: Investigation of a family after a fatal case. 119 61

Procaine caused respiratory arrest in halothane-anaesthetized normal Landrace pigs at a dose of 4,2 - 8,2 mg/kg. In some cases death due to respiratory failure occurred after giving 10 - 13 mg/kg. Because of its toxicity in pigs procaine is unsuitable for treating the porcine malignant hyperthermia syndrome.
J S Afr Vet Assoc 1975 Sep
PMID:On the toxicity of procaine for pigs. 121 6

In vitro contracture tests for susceptibility to malignant hyperthermia (MH) were performed in 96 patients according to the protocol of the European MH Group. In addition, tests were performed with halothane 0.44 mmol l-1 and 0.66 mmol l-1, and caffeine 2 mmol l-1, each added as a single bolus dose to fresh specimens. For all tests the size of contractures were recorded, and for the diagnostic tests the halothane and caffeine threshold concentrations were determined (i.e. the minimal concentrations eliciting a contracture of 0.2 g). The caffeine specific concentration (CSC, i.e. the concentration increasing force 1.0 g) and the % increase with caffeine 2 mmol l-1 were calculated from the dose response curves. Various diagnostic criteria in use by the North American MH Group were applied, and diagnostic outcome compared with the result obtained by the protocol of the European MH Group. Thirty-five patients were susceptible to MH (MHS), 33 were non-susceptible (MHN), and 28 had equivocal results of the tests (MHE). Additional tests were made in 34 MHS, 32 MHN, and 26 MHE patients. Contractures elicited by bolus addition of halothane or caffeine were significantly larger than those observed following the same dose of drug added cumulatively (P less than 0.05). Contractures greater than or equal to 0.7 g following halothane 3% (bolus dose) were seen in 78% of MHS patients and 18% of MHN patients, A CSC less than 4 mmol l-1 was elicited in 86% of MHS and 30% of MHN patients, whereas an increase in force greater than or equal to 4% or greater than or equal to 7% was seen in 71% and 34% of MHS patients, respectively, and in none of the MHN patients. Using the criterion of greater than or equal to 0.7 g in the halothane test and greater than or equal to 4% increase in the caffeine test gave the best agreement between diagnostic outcome with the European and North American protocols: All 34 MHS patients (100%) were positive to one or more tests, but so were eight of 32 MHN patients (25%), giving an overall diagnostic agreement of 88%. We conclude that, in our laboratory, the results obtained with the two major protocols for investigation of MH susceptibility are not identical. Patients surviving fulminant MH, however, react abnormally to nearly all the tests. For validation and possibly further standardization of the tests each laboratory must investigate a large number of normal controls and as many patients surviving fulminant MH as possible.
Eur J Anaesthesiol 1992 Sep
PMID:Sources of variability in halothane and caffeine contracture tests for susceptibility to malignant hyperthermia. 139 23

In vivo muscle 31P nuclear magnetic resonance spectroscopy was performed on 12 homozygous halothane-nonsensitive female pigs and 13 female pigs heterozygous with respect to the halothane gene. Fifteen female pigs of a third line, consisting of heterozygotes and halothane-nonsensitive homozygotes, were also available. Body weight ranged from 12 to 18 kg. Mean decrease in phosphocreatine concentration in the biceps femoris of anesthetized pigs was significantly lower for heterozygous vs homozygous pigs (3.46% vs 5.94%, P less than 0.01) after 40 minutes of halothane exposure (3%; oxygen flow, 3 L/min). Also, a statistically significant difference, with respect to the initial (7.21 vs 7.11, P less than 0.008) and end muscle pH values (7.18 vs 7.06, P less than 0.0002), was observed for homozygous vs heterozygous pigs. By means of canonical discriminant analysis, it was possible to distinguish nonsensitive homozygotes from heterozygotes (P less than 0.0001). When applying this classification method to pigs of the same strain, 2 populations (nonsensitive homozygotes, heterozygotes) emerged, with a proportion of pigs corresponding to the expected value on the basis of breeding records. In contrast to the phenotypic expression of muscular rigidity related to the malignant hyperthermia syndrome, the expression of metabolic variables (phosphocreatine, pH) was shown to be dominant.
Am J Vet Res 1992 Sep
PMID:Identification of halothane gene carriers by use of in vivo 31P nuclear magnetic resonance spectroscopy in pigs. 834 46

The levels of serotonin (5-HT) were determined in platelet-free and platelet-rich plasma before and during the onset of halothane-induced malignant hyperthermia (MH) in genetically MH-susceptible pigs. During MH onset, the free (i.e. physiologically active) levels of 5-HT in plasma rose concomitantly with the increases in muscle tone, body temperature, venous pCO2 and plasma lactate. Since pharmacological stimulation of 5-HT2 receptors has recently been shown to trigger MH in susceptible pigs, the finding of increased 5-HT plasma levels during onset of halothane-induced MH may indicate that 5-HT is involved in the mechanisms by which volatile anesthetics trigger this myopathic syndrome.
Eur J Pharmacol 1992 Sep 10
PMID:Increase of serotonin in plasma during onset of halothane-induced malignant hyperthermia in pigs. 142 84

Children otherwise suitable for same-day discharge may be admitted to the hospital solely because they are known or suspected to be malignant hyperthermia-susceptible (MHS). To determine whether their hospitalization is necessary, the medical charts of 303 children labeled MHS who had undergone surgery with anesthesia free of malignant hyperthermia-triggering agents on 431 occasions between 1981 and 1990 were reviewed. Eighteen of these patients (25 cases) who were subsequently identified as biopsy-negative were excluded from the study. We recorded the reason for the MHS label and the perioperative management and outcome of the cases. Fifty-eight percent of procedures were followed by hospital admission solely because of the patient's MHS label. None of the 25 children (33 cases) with biopsy-proven malignant hyperthermia developed intraoperative or postoperative pyrexia. Ten children suspected to be MHS developed pyrexia greater than 38.5 degrees C. These episodes were not considered to be malignant hyperthermia and were not treated with dantrolene. None of the remaining 275 patients exhibited any features of malignant hyperthermia, although one had an adverse reaction to radiologic contrast medium. On the basis of our retrospective analysis, postoperative admission to the hospital solely on the basis of the MHS label is not warranted.
Anesth Analg 1992 Sep
PMID:Should all children with suspected or confirmed malignant hyperthermia susceptibility be admitted after surgery? A 10-year review. 151 Feb 54

A point mutation in the human gene for the skeletal muscle calcium release channel (ryanodine receptor [RYR1]) correlates with inheritance of malignant hyperthermia in a family of Northern European descent. The substitution of thymine for cytosine at position 1840 of the RYR1 transcript results in a cysteine-for-arginine substitution at position 614 (R614C) of the amino acid sequence. The mutation was absent in 59 normal individuals from the general population, in 61 additional unrelated malignant hyperthermia-susceptible patients, and in 18 patients with malignant hyperthermia associated with other inherited or congenital diseases. Together with reports of an equivalent mutation in six susceptible pig strains and an identical mutation in one other human pedigree, these findings suggest that the cysteine-for-arginine mutation represents a shared calcium release channel pathogenesis between porcine malignant hyperthermia and a subset of mutations responsible for the human malignant hyperthermia syndrome.
Anesth Analg 1992 Sep
PMID:A cysteine-for-arginine substitution (R614C) in the human skeletal muscle calcium release channel cosegregates with malignant hyperthermia. 151 Feb 67


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