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)

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

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

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.
Anaesthesia 1975 May
PMID:Treatment of porcine malignant hyperthermia. A review based on experimental studies. 23 11

Malignant hyperthermia is a dominantly inherited, usually subclinical, disease that occurs in individuals who have an underlying muscular disorder and connotes the gravest possible consequences. When it occurs, it is usually during the use of muscle relaxants in anesthesia and potent anesthetic agents such as halothane. Patients at risk must be identified through careful history and screening procedures; however, a patient susceptible to this condition may have had general anesthesia in the past without complications. A careful monitoring regimen must be established for the procedure and some means of cooling the patient must be ready in case pyrexia occurs. Dantrolene sodium is currently the preferred drug for prevention of the syndrome and may be valuable for its treatment.
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PMID:Dantrolene sodium in the management of patients at risk from malignant hyperthermia. 28 34

Systemic hyperthermia in man may occur by accident, as in heat stroke or malignant hyperthermia during general anesthesia, or it may be therapeutically induced (fever therapy). The latter has been used infrequently since the advent of antibiotics, except recently for treatment of cancer. Local or regional heating combined with x irradiation for human cancer therapy has been sporadically reported for over 60 years, but has not found its place in clinical medicine possibly due to technical limitations in heat production and dosimetry. Preliminary results are reported for treatment of spontaneous animal tumors with radiofrequency current fields and x irradiation.
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PMID:Prospects for hyperthermia in human cancer therapy. Part I: hyperthermic effects in man and spontaneous animal tumors. 32 7

Malignant hyperpyrexia is a genetically related syndrome that cannot be predicted reliably in advance of administration of anesthesia except by a strong family history. The definitive etiology is unknown, although triggering agents that release calcium from the calcium-storing sarcoplasmic membrane of the muscle cell are highly suspect. As soon as the syndrome is diagnosed, therapy must be prompt, vigorous, and carried out with the same urgency as a cardiac arrest. Specific therapy with dantrolene sodium may prove to be an answer to this serious problem in anesthetic practice.
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PMID:Malignant hyperpyrexia. 32 54

A case of pregnancy complicated by malignant hyperthermia susceptibility is reported. Serum CPK and electrolyte concentrations were measured during pregnancy and labour. Labour and delivery were managed successfully under epidural analgesia using plain bupivacaine 0.5%.
Anaesthesia 1979 Jan
PMID:Malignant hyperthermia susceptibility. Management during pregnancy and labour. 42 40

The use of procainamide or procaine for treatment of malignant hyperthermia is commonly recommended. The skeletal muscle relaxant dantrolene has also been indicated for treatment of this complication during anesthesia. In the present study, effects of procainamide and dantrolene were compared in malignant hyperthemia-susceptible (MHS) pigs in vivo and on MHS muscle from human patients in vitro. The ED50 for dantrolene block of indirectly evoked twitch tension was 0.85 mg/kg in MHS pigs. A final cumulative dose of 2 mg/kg resulted in 68 per cent block of the twitch response. In contrast, procainamide at a final cumulative dose of 14 mg/kg had no effect on twitch response of the MHS pigs. Dantrolene, 3 micrometer, in vitro (approximately 0.8 mg/kg in vivo) was effective in preventing or reversing the abnormal halothane-induced contracture response of human MHS muscle strips. Procainamide, 0.11 mM, a dose approximating clinical levels (about 22 mg/kg), had no effect on basal twitch response or on the abnormal halothan-induced contracture of MHS human muscle. These results confirm the effectiveness of dantrolene and the lack of effectiveness of procainamide in the treatment of malignant hyperthemia.
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PMID:Rationale for dantrolene vs. procainamide for treatment of malignant hyperthermia. 43 84

The essential epidemiologic, pathophysiologic and clinical data of malignant hyperthermia are presented. Prophylactic measures, early recognition and an effective therapy schedule may reduce the appallingly high lethality of this rare complication during general anaesthesia.
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PMID:[Malignant hyperthermia: clinical aspects and treatment (author's transl)]. 45 50


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