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)

Although consideral information is available concerning the structural and biochemical changes in the skeletal muscles of patients with malignant hyperthermia, little is known of the cardiac changes in this disease. However, ventricular fibrillation and cardiac arrest are frequent in these patients. In 3 patients with malignant hyperthermia, contraction bands and foci of myofiberlysis were found in the heart at necropsy. Ultrastructurally, areas of myofiber overstretching adjacent to contraction bands and foci of extensive myofiberlysis were associated with disruptions of the sarcolemma. Similar ultrastructural findings have been reported in the skeletal muscles of these patients and are thought responsible for the hyperkalemia which is a constant feature of malignant hyperthermia. Our findings suggest that the ventricular arrhythmias, frequent in this disease, are the result of direct damage to cardiac muscle rather then secondary to elevated plasma levels of potassium.
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PMID:Myocardial changes in malignant hyperthermia. 33 38

Human endothelial cell monolayers prepared from umbilical veins have been incubated with aspirin (1--2 mM) dissolved in Hepes modified solution and in platelet-rich plasma. They have also been incubated with plasma prepared from subjects before and after intake of aspirin giving a mean plasma concentration of 0.5 mM. The effects of the endothelial cells on ADP and collagen-induced platelet aggregation and malondialdehyde production in platelet-rich plasma have been tested. The endothelial cells had a spontaneous inhibitory effect on all three parameters. This effect was abolished when the cells were incubated with aspirin dissolved in MHS for 20 min and the increase in effect observed when platelet-rich plasma was incubated with endothelial cells for a period of 30 min was similarly inhibited when aspirin was dissolved in plasma or when plasma prepared from subjects who had taken aspirin were used. Aspirin had no inhibitory effect on prostacyclin (PGI2) with regard to the effect of PGI2 on platelets. On the contrary, the two compounds had an additive inhibitory effect on platelet aggregation induced by ADP and collagen. These findings should be considered with regard to the use of aspirin as an antithrombotic agent.
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PMID:The inhibitory effect of aspirin on human endothelial cells. 36 62

A short review of the history, incidence, and present-day concept of the etiology of malignant hyperthermia is presented. Protocols for diagnosis, treatment of the unexpected occurrence, and management of known malignant hyperthermic patients is presented. The exact etiology of the syndrome is still unknown.
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PMID:Malignant hyperthermia. 39 59

Malignant hyperthermia may appear during surgery. It has at least three features: 1) an anesthesiological trigger, usually the association of halothane and succinylcholine; 2) rapid increase in body temperature; 3) widespread muscle hypertonia. The literature is reviewed in an assessment of the physiopathological mechanism underlying the syndrome, with particular reference to the part played by calcium. Experimental data are cited and their similarity with the clinical, laboratory, anatomical, and histopathological picture in man is discussed. A detailed account is also given of two personal cases. Lastly, questions associated with the prevention and treatment of malignant hyperthermia are examined.
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PMID:[Malignant hyperthermia. Review of literature and case reports]. 39 55

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%.
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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

In vitro muscle contracture responses in swine susceptible to malignant hyperpyrexia (MH) were similar to those found in muscle from humans susceptible to this anaesthetic complication, confirming the suitability of the pig as an animal model for studying MH. The results suggest that there are different degrees of susceptibility to MH. Whichever drug was used, there was some overlap in the contracture responses between susceptible animals and controls, suggesting that the most accurate way of identifying susceptibility to MH is to use a variety of chemical agents, the best of which seem to be halothane, caffeine, suxamethonium and potassium chloride. Thymol, which is used as a preservative in commercial preparations of halothane, potentiates halothane contractures, but it is not known if this is significant clinically.
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PMID:Identification of susceptibility to malignant hyperpyrexia in swine. 43 38

In a series of 53 fenfluramine intoxications (15 taken from the literature), 10 were lethal after doses of 28.7--70 mg/kg of body weight. Cardiac arrest occurred 1--4 hr after ingestion in 9 cases; all these 9 patients died. Two out of 3 patients with more than 15 mg/kg had coma and convulsions. Other frequent signs were mydriasis, tachycardia, and rubor of the face. The additional signs of nystagmus, hypertonia, trismus, hyperreflexia, clonus, excitation, hyperthermia, and sweating define the clinical syndrome of fenfluramine intoxication. Symptoms begin 30--60 min after ingestion and can persist during several days. Early gastric lavage, instillation of activated charcoal, diazepam in case of seizures, chlorpromazine for malignant hyperthermia, propranolol for extreme tachycardia, and lidocaine in the event of ventricular extrasystoles are recommended. If trismus is a prominent sign, muscle relaxants must be given before gastric lavage can be done. The relatively benign course after survival of the first 4 hr suggests supportive therapy only in the later phase of intoxication.
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PMID:Fenfluramine poisoning. 43 87

The muscle biopsy from a 12-year-old boy with chronic creatine kinase elevation was studied by electron microscopy. At the age of 7 years the patient had a possible abortive attack of malignant hyperthermia. The biopsy specimen contained many fibres with segmental contracture and necrosis; thin sections showed defects of the plasma membrane with loss of glycogen granules into the interstitium. Freeze-fracturing of the plasma membrane showed clustering of intramembranous particles and some membrane areas were devoid of particles and of pinocytotic caveolae. The E-face showed irregular elevations and the P-face corresponding defects. These deviations of the fracture-plane were due to manifest membrane openings, to abnormalities of the structure of the lipid bilayer, to clustering of membrane particles or to the fact that intracellular membranes often were abnormally close to the plasma membrane. The findings suggest that a disintegration of the lipid-protein system of the membrane precedes the formation of manifest defects. The manifest defects resembled those in Duchenne muscular dystrophy. It is unknown whether the changes seen by freeze-fracturing were specific for a myogenic disorder and whether they were due to a basic membrane abnormality or to defects in other systems of the muscle cell.
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PMID:A freeze-fracture study of the plasma membrane of muscle fibres of a patient with chronic creatine kinase elevation suspected for malignant hyperthermia. 44

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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