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

12 healthy persons--close relatives to two children who died of malignant hyperthermia--volunteered for biopsies of striated muscle and skin, electromyography and electroencephalography. The investigations of muscle biopsies comprised material for enzyme histochemistry and ordinary light microscopy including visualization of the intramuscular nerves. Out of 12 clinically healthy persons 9 revealed abnormalities of the muscle fibres, 11 showed degenerative and regenerative alterations in the intramuscular nerves, in EMG 7 turned out to produce slight neuropathy, and 7 displayed abnormal EEG tracings. These findings support the idea that the etiological key (or keys) in the peculiar pathophysiological entity of malignant hyperthermia may even be found outside the striated muscle cell.
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PMID:Malignant hyperthermia in a family. The neurophysiological and light microscopical study of muscle biopsies of healthy members. 12 7

Four carriers of the abnormal trait for malignant hyperpyrexia have been studied to determine the value of the various methods of carrier detection. Tests included enzyme estimations, histochemistry, electronmicroscopy, biochemistry and in vitro exposure of muscle strips to halothane. By combining these procedures it is possible to detect all the asymptomatic carriers of this disease.
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PMID:Predictive tests for malignant hyperpyrexia. 12 73

The presence of denervation-like changes and abnormal mitochondria in the muscle of carriers of the malignant hyperthermia syndrome suggest a neuropathic basis for the human syndrome. A defect in mitochondrial ATP synthesis resulting from denervation, and potentiated by some general anaesthetics, may be the primary muscle fibre lesion in the human malignant hyperthermic syndrome.
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PMID:Malignant hyperthermia syndrome--evidence for denervation changes in human skeletal muscle. 13 24

Vastus lateralis muscle was excised from normal pigs and from pigs susceptible to malignant hyperthermia. Anesthesia consisted of diazepam, N2O:O2, and a lidocaine field block. In the susceptible (MHS) pigs, respiratory control in mitochondria of excised muscle was normal, while electron transport was accelerated. Glutamate log Q3's and log Q4's were in inverse linear relationship to porcine weights. In the presence of glutamate, state 3 respiration was 3.5 times, and state 4 respiration 3.2 times, higher in MHS than in normal mitochondria (independent of weight or halothane dose). Thus, the MHS mitochondria were able to produce ATP more efficiently than normal mitochondria. In vitro, halothane inhibited glutamate Q3's and R.C.I.'s, slightly increased succinate Q3's and R.C.I.'s, had no significant effect on glutamate or succinate Q4's, and, moderately lowered glutamate and succinate P/O ratios. These changes were similar to those observed in normal pigs. Calcium uptake into MHS mitochondria was markedly less than normal but was not significantly altered by in-vitro halothane. These results suggest a mitochondrial membrane component for the defect of procine malignant hyperthermia since calcium is normally associated with the formation of the phospholipid lattice of this membrane. (Key words: Hyperthermia, malignant; Metabolism, mitochondrial; Muscle, skeletal, mitochondria; Anesthetics, volatile, halotbane; Ions, calcium.)
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PMID:Porcine malignant hyperthermia: effects of halothane on mitochondrial respiration and calcium accumulation. 16

Pituitary-adrenal function was investigated in two groups of Pietrain pigs differing by their susceptibility to the malignant hyperthermia syndrome induced by halothane, and in Large White pigs (LW). Plasma basal levels of glucocorticoids, measured by a protein binding technique, and plasma ACTH concentrations, determined by radioimmunoassay, did not differ according to halothane susceptibility but were higher in LW pigs compared with Pietrain pigs. The reactivity of the pituitary-adrenal axis to such stressors as exposition to a novel environment or shaking was the same for all experimental groups. The same was true concerning plasma levels of glucocorticoids and ACTH after injection of dexamethasone and response of the adrenal cortex to a standard dose of ACTH. These results demonstrate that LW pigs have a higher tonic secretion of ACTH than Pietrain pigs, but with identical phasic response to stimulations. Halothane susceptibility by itself appears to have no influence of pituitary-adrenal function.
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PMID:Behavioural and pituitary-adrenal characteristics of pigs differing by their susceptibility to the malignant hyperthermia syndrome induced by halothane anesthesia. 2. Pituitary-adrenal function. 21 55

The effects were investigated of a 25-minute inhalation of halothane with oxygen on three to four months old pigs of the Large White breed. Symptoms of malignant hyperthermia did not occur. The actual total anesthesia, which causes slight hypoproteinemia, hypoglycemia and hypocholesterolemia without significant changes in the content of non-esterified fatty acids (NEFA) and urea, induced only a slight increase of circulating 11-hydroxycorticosteroids (11-OHCS). The combination of anesthesia with castration of gilts or barrows significantly increased the concentration of 11-OHCS but did not reach the level recorded after the application of ACTH. The higher levels of 11-OHCS were accompanied by higher concentrations of NEFA and glucose. The treatment of the animals lasting half an hour prior to inhalation of halothane at maximum doses or one hour in the control unanesthetized pigs produced an effect, mainly on the 11-OHCS concentration and on the activity of creatine kinase in the plasma. The results indicate that the adrenocortical response to the effect of halothane is not stronger than the response to simple handling connected with excitement and muscular activity of the animals. Therefore there is no reason of considering halothane anesthesia as a factor causing great stress and pigs which in its course do not respond with malignant hyperthermia as animals insensitive to stress. The aptness of denotation of clinical manifestations of genetically defective muscles in pigs is discussed.
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PMID:[The effect of halothane anesthesia on the function of the adrenal cortex and some metabolites in the blood plasma of pigs not susceptible to malignant hyperthermia]. 22 19

Virus-like particles (VLP) have been identified by electronmicroscopy in the skeletal muscles (paraxials) of six cases of idiopathic scoliosis. These particles closely resembled VLP reported in the skeletal muscles in other conditions, e.g. Reye's syndrome, polymyositis, malignant hyperthermia, and chronic myopathy. We have shown by specific staining that these structures are composed of glycogen in a crystalline form. Using Coxsackie B infected tissue culture cells as a control we have shown that these viruses, which are of similar shape and size to the VLP, were unstained using this specific staining method.
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PMID:The nature of virus-like particles in the paraxial muscles of idiopathic scoliosis. 23 Mar 31

Enzymatic properties of erythrocyte membranes in Duchenne muscular dystrophy (DMD) and malignant hyperthermia (MH), two genetically determined abnormalities of skeletal muscle, were examined. Acetylcholinesterase (AChE) and ATPase activities were chosen for investigation since alterations in these enzymes have been demonstrated in animal models of dystrophy. A significant decrease in Na+,K+-ATPase activity was noted in DMD patients and a number of possible DMD carriers, suggesting that this enzyme may provide a useful marker of the carrier state in carriers not exhibiting an elevation in plasma creatine phosphokinase activity. No abnormalities in AChE were demonstrable in any of our DMD patients, indicating that human dystrophy is biochemically distinct from certain animal models of dystrophy (e.g., dystrophic mice) where erythrocyte AChE is decreased. In contrast, evidence was found in two known MH carriers, who had normal erythrocyte ATPase activities, for the presence of an altered membrane AChE characterized by an increase in substrate affinity and a large decrease in maximal hydrolytic rate. While the exact relevance of this membrane defect, if any, to the pathogenesis of MH remains to be seen, the presence of this modified enzyme may serve to identify those individuals in a family where a positive history of MH exists who are at risk of developing a hyperthermic crisis during anesthesia.
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PMID:Erythrocyte membrane enzyme abnormalities in two hereditary disorders of muscle. 23 Oct 77

The metabolic and endocrine changes in porcine malignant hyperthermia are described and the importance of the catecholamine response emphasised. The relative effectiveness of several different therapeutic agents in the treatment of this syndrome in the Pietrain pig are described and the importance of a common link with the catecholamine response discussed.
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PMID:Treatment of porcine malignant hyperthermia. A review based on experimental studies. 23 11

MHS and normal pigs were anaesthetized with nitrous oxide and diazepam. Halothane 1% was then administered for 1 hr. Immediately before and immediately after the halothane inhalation, 10 g of vastus lateralis muscle was excised. SR was isolated from this muscle. Calcium accumulation by the MHS sarcoplasmic reticulum in the absence of halothane was greater than normal. Halothane in vitro produced a similar increase in calcium uptake into both the MHS and the normal SR. Halothane in vivo significantly reduced calcium accumulation by hyperthermic SR but had no significant effect on calcium uptake into the normal SR. Thus the action of halothane on the MHS sarcoplasmic reticulum is indirect, occurring only in the intact cell. It is possibly a result of the deleterious effect which lack of ATP, a low pH or a high temperature is likely to have on the calcium accumulating mechanism of the SR membrane. Our results suggest that the primary defects of porcine and human malignant hyperthermia are not entirely synonymous.
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PMID:Calcium uptake into muscle of pigs susceptible to malignant hyperthermia: in vitro and in vivo studies with and without halothane. 23 50


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