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)

A member of a family which was known to be susceptible to malignant hyperpyrexia, who was identified as a carrier by the presence of an elevated serum creatine-phosphokinase, has been investigated further. Muscle was examined biochemically, and the study included the sarcoplasmic ATPase-activity, actinomycin, Mg2+ ATPase activity, ATP, phosphocreatine and glucose-6-phosphate. In addition, the calcium uptake by the sarcoplasmic reticulum was studied. The histochemical analysis of the muscle revealed the presence of a new fibre type characterised by a dense rim of ATPase activity, which gives the impression of a 'picture-frame'. Ultramicroscopic study revealed changes in the mitochondria and areas of myofibrillar disruption with swelling of the sarcoplasmic reticulum.
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PMID:'Picture frame' fibres in a carrier of the trait for malignant hyperpyrexia. 0 Jul 95

Metabolic, hemodynamic and neuroendocrine responses to the combined use of halothane and succinylcholine (SCh) were measured in five normal swine and five swine susceptible to malignant hyperthermia (MH). Constant-volume ventilation was used, and no therapy was instituted. The overall response in susceptible swine was fulminant, in that it involved the rapid onset of SCh-induced MH combined with the more severe metabolic, endocrine, and cardiovascular effects of halothane-induced MH. Maximal changes in VO2 were equivalent with either drug or both combined, while changes in lactate, potassium (K+), pH, and catecholamines were perhaps synergistic. Utilizing similar measurements, procaine or procainamide was used in 20 susceptible swine in attempts to prevent MH initiated by halothane, SCh, or both. Recommended therapeutic doses of either drug did not prevent characteristic MH changes in oxygen consumption, cardiac output, lactate, K+, pH, catecholamines, or temperature.
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PMID:Porcine malignant hyperthermia induced by halothane and succinylcholine: failure of treatment with procaine or procainamide. 0 27

The effects of the establishment of an adrenergic blockade on suxamethonium-induced porcine malignant hyperthermia (MH) were investigated in Pietrain pigs. Six animals were fed reserpine 10 mg daily for 7 days and then challenged with suxamethonium. Three survived but the remainder developed fatal MH. In a further study of 10 pigs, either phentolamine 40 mug/kg/min or propranolol 50 mug/kg/min were administered for 30 min before suxamethonium stimulation and continued for the duration of the experiment. The five year beta-blocked pigs all became hyperthermic and died whereas the phentolamine-treated group survived. However, both alpha adrenergic blockade and successful reserpinization failed to prevent the abnormal muscle response to the first dose of suxamethonium.
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PMID:Porcine malignant hyperthermia. III: Adrenergic blockade. 0 23

This study concerns 3 cases of malignant syndrome due to neuroleptics. Our aim is to clarify the nature of this syndrome and to attempt to delimit it from malignant hyperthermia. The etiology suggests that its occurence should increase with the growing use of very active neuroleptics. It consists clinically of an extrapyramidal syndrome with hypertonicity, akinesia, tremor and with a neuro-vegetative syndrome of which the main sign is hyperthermia. On the basis of physiopathology it is attempted to explain, in the light of fresh data, the extrapyramidal signs, the hyperthermia and the rise in the OPK Etiological treatment has, however, proved disappointing.
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PMID:[Malignant syndrome caused by neuroleptics (3 observations)]. 3 12

Strandard laboratory rabbits which are not genetically susceptible to malignant hyperpyrexia were anesthetized with either halothane or pentobarbital. Administration of caffeine in 125 mg increments produced a syndrome strongly resembling malignant hyperpyrexia in rabbits anesthetized with halothane. All these animals became rigid, hyperpyrexic, acidotic and hyperkalemic, whereas caffeine-treated, pentobarbital-anesthetized animals developed only mild acidosis. Pentobarbital alone and halothane alone caused no changes in measured variables. This model for malignant hyperpyrexia resembles the naturally occurring disease more closely than several preceding pharmacologic models.
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PMID:A laboratory animal model for malignant hyperpyrexia. 3 78

The purpose of this investigation was to see whether or not the hypothetical disease susceptibility locus to ankylosing spondylitis is linked to the MLC determinant 27a. Firstly, we discovered a strong MLC determinant which we called 27a because of its association with the second series specificity W27. Later, we found that this determinant was the same as that which Jorgensen et al. (1973) found to be associated with the second series specificity W5. These MLC determinants may, therefore, be in linkage disequilibrium with more than one HL-A specificity (in this case, of the second series) as previously described by Dupont et al. (1973). However, we found no association between 27a and ankylosing spondylitis. On the other hand, we confirmed the association of ankylosing spondylitis with W27 and also found an increase of HL-A2 of the first series which, however, was not statistically significant. In view of the high incidence of HL-A2 in the general population, more information is required to definitely establish such an association. We found no special association with specificities AJ and Hu (Sa 532), third series antigens, but confirmed their linkage disequilibrium with W27. Disease predisposition loci seem, therefore, to be associated with either type of MHS marker, multiple sclerosis and MLC determinant 7a, or ankylosing spondylitis and the second series antigen W27. It will be interesting to discover whether there is any special significance of the association of one marker rather than another.
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PMID:Ankylosing spondylitis and the major histocompatibility system. 4 91

The frequency of HL-A8 in myasthenia gravis is markedly increased in women (60-80%) but not in men. The MLC determinant, LD-8a, is frequently associated with HL-A8. Of the 37 female MS patients, 15 were LD-8a positive (41%), whereas of the males only one of seven was LD-8a positive. The frequency of HL-A8 was 68% in women and 29% in men with the disease. We therefore conclude that the gene which is responsible for the increased susceptibility to myasthenia gravis in women and which is present in the MHS region, is more closely linked to the SD-2 than to the LD-1 locus.
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PMID:HL-A8 and LD-8a in patients with myasthenia gravis. 5 59

By the halothane test we determined the development of the syndrome of malignant hyperthermia in pigs of the Belgian Landrace breed with body weight of 20 kg. We found that from 27 animals that were subjected to the test 12 positively reacted to Narcotan - Spofa anaesthesis. Pigs with the syndrome of malignant hyperthermia had considerably higher rectal temperature after anaesthesis, lower PH of venose blood, higher concentration of lactic acid and glucosis in the blood plasma. Halothane-negative animals did not produce and conspicuous changes of these indices. Most differences were statisticaly highly significant.
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PMID:[Utilization of the halothane test in the detection of the malignant hypertension syndrome [MHS] in pigs]. 9 39

The intensity of the mixed lymphocyte reaction to H-2-linked mixed lymphocyte reaction stimulating genes is influenced by non-H-2 genotype. The same H-2 incompatibility results in a stronger mixed lymphocyte reaction when both partners have the C57BL/10ScSnPh genetic background than when they have the genetic background of the A/Ph strain. It is suggested that, as in man, the intensity of the mixed lymphocyte reaction to incompatibility at the MLR-S genes linked to the major histocompatibility complex is controlled by MLR-response genes not linked to MHS.
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PMID:Influence of non-H-2 genotype on the response to H-2-linked mixed lymphocyte reaction stimulating (MLR-S) genes. 12 14

This communication presents evidence in support of a neuropathic basis for the myopathy associated with malignant hyperpyrexia (MH). Muscle from MH susceptible individuals showed a reduced calcium uptake by the sarcoplasmic reticulum. There was a reduced concentration of phosphocreatine and ATP and an increased concentration of glucose-6-phosphate in these muscle samples.
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PMID:Morphological and biochemical defects in muscles of human carriers of the malignant hyperthermia syndrome. 12 80


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