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

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

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 clinical and laboratory findings in 4 children with signs of malignant hyperthermia are reported. In all cases an extraordinary elevation of creatine kinase activity in serum was observed. By investigation of the creatine kinase isoenzyme activities we tried to determine the origin of creatine kinase. In contrast to other reports, creatine kinase BB derived from brain was found to be absent in all cases, although creatine kinase MM and MB showed remarkable alterations. A certificate for all patients who have survived malignant hyperthermia is suggested.
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PMID:[Clinical and laboratory aspects of malignant hyperthermia in children with special reference to creatine kinase isoenzymes (author's transl)]. 64 84

Malignant hyperthermia (MH) is a pharmacogenetic disorder of skeletal muscle. In genetically susceptible pigs, MH can be induced by volatile, halogenated anaesthetics such as halothane. Within a series of pharmacological investigations, a fulminant MH could be induced in 59 of 66 homozygous halothane-susceptible pigs by a challenge with 3% halothane for 15 minutes. The typical MH was characterized by sudden appearance of tachycardia, muscle rigidity with typical extension of the hindlimbs, increase of body temperature, acidosis-caused by rapid increase of CO2 and lactate production-, hyperkalaemia and increased activity of creatine kinase (CK) and aspartate transaminase (AST). In seven homozygous MH-susceptible pigs, this typical MH could not be induced by halothane. These animals responded with sudden appearance of bradyarrhythmia and decrease of arterial pressure. In these MH-atypical pigs (MHA) neither the typical extension of hindlimbs nor a hyperthermia occurred. Compared to a group of 6 MH-susceptible pigs with typical reactions to halothane (MHS), the biochemical alterations were significantly retarded in MHA-pigs. These atypical reactions to halothane could be the effect of decreased cardiac output. Concerning the atypical reactions, we observed a familiar predisposition in MH-susceptible pigs. Although atypical reactions were not found in a group of homozygous halothane-nonsusceptible pigs (MHN), a possible explanation for atypical reactions could be a MH-independent halothane-susceptibility of the myocardium+ in MHA-pigs. On the other side the data may indicate that a primary defect in both the skeletal muscle and also the myocardium is involved in MH. The different reactions to halothane in MH-susceptible pigs could point to a genetic heterogeneity.
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PMID:Atypical reactions to halothane in a subgroup of homozygous malignant hyperthermia(MH)-susceptible pigs: indication of a heterogenous genetic basis for the porcine syndrome. 142 16

Malignant hyperthermia (MH) is a rare clinical syndrome characterized by hypermetabolism and triggered by specific anesthetic agents. The mechanism of this abnormal reaction is due to uncontrolled calcium flux in the skeletal muscles resulting in a variable clinical syndrome of muscle rigidity, respiratory and metabolic acidosis, and elevation of temperature. The specific genetic defect underlying this condition has not been identified in humans, though in susceptible swine a mutation of the gene for the ryanodine receptor, a large protein which comprises the calcium channel in the sarcoplasmic reticulum, has been identified recently. Inheritance in humans appears to be autosomal dominant with variable penetrance. Patients with MH rarely have physical or laboratory signs of muscle disease. However, scattered case reports and investigations of individuals with known myopathies and other muscle related problems, such as acute rhabdomyolysis or idiopathic persistently elevated creatine kinase, suggest a possible association of MH with a variety of neuromuscular diseases and stress syndromes. This association is very strong in the case of central core disease (CCD) where it is supported by clinical and laboratory evidence, including the proximity of the CCD gene to the ryanodine receptor gene on chromosome 19. A variety of other diseases have been implicated and can be classified as possibly associated (King-Denborough syndrome, Duchenne muscular dystrophy) or unlikely to be associated (myotonia congenita, sudden infant death syndrome, limb girdle dystrophy, neuroleptic malignant syndrome, etc.).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Malignant hyperthermia and neuromuscular disease. 148 40

The authors report on the course of a fulminant malignant hyperthermia (MH) associated with laminectomy in a 29-year-old man who had been healthy up to that time. Succinylcholine and isoflurane were considered to be the causative triggering agents. Progression could be prevented due to an early suspicion raised by end-expiratory CO2 measurement: treatment was instituted immediately (Dantrolene 2mg/kg body weight, oxygen hyperventilation, external cooling, etc.) Serum creatine kinase increased up to almost 50,000 U/l associated with massive myoglobinuria. Residue-free restitution was achieved within a few days. Decisive for an early detection of MH is the routine performance of end-expiratory CO2 measurement which is definitely superior to temperature control and significantly reduces the time that elapses before treatment is initiated.
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PMID:[The early diagnosis of malignant hyperthermia--the place of end-expiratory CO2 monitoring]. 178 8

This retrospective study was undertaken to examine the management and outcome of children who developed isolated masseter muscle spasm (MMS) after the administration of intravenous succinylcholine during anesthetic induction. The inhalation anesthetics used for induction were continued in all of these cases. The medical records of 68 patients (male/female ratio, 1.7:1), identified from approximately 42,000 anesthetics given during the period 1980-1989, were reviewed. Fifty-seven children (2.3-12 yr old) were diagnosed as having isolated MMS, i.e., MMS without spasm of other muscles; 11 experienced generalized rigidity in combination with MMS. Anesthetic and postoperative management of these two groups differed. The overall incidence of MMS was 0.3% of inhalation anesthetics during which succinylcholine was given. Intraoperative arrhythmias occurred in 33% of the patients who developed isolated MMS and more frequently in older children. Most children experienced some degree of hypercarbia and/or metabolic acidosis, but the significance of these abnormalities in the spontaneously ventilating, fasting child is unknown. Serum creatine kinase levels when measured 18-24 h postoperatively were elevated in all but one child (n = 45). There was no long-term morbidity and no mortality. We conclude that failure of the masseter muscles to relax after succinylcholine is not uncommon in children. Based on our experience, and accepting that MMS may be part of the clinical spectrum of malignant hyperthermia, we believe that anesthesia can be continued safely in cases of isolated MMS when careful monitoring accompanies diagnostic evaluation. This differs from the current practice of discontinuing the anesthetic or switching to a nontriggering anesthetic technique.
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PMID:Masseter muscle spasm in children: implications of continuing the triggering anesthetic. 186 39

This report describes a cardiac arrest that occurred in a 4-month-old infant during induction of anesthesia. During the administration of N2O/O2 and halothane via a face mask tachycardia was noted and rigor followed the application of succinylcholine for intubation. Shortly thereafter cardiac arrest occurred; 15 min later we found a profound metabolic acidosis as well as signs of rhabdomyolysis with a serum potassium level of 10.3 mmol/l and an increase in serum creatine kinase (CK). While performing cardiopulmonary resuscitation (CPR) and treating the acid-base imbalance and hyperkalemia, we administered--suspecting malignant hyperthermia (MH)--dantrolene. Approximately 60 min post-arrest we achieved stabilization of the vital signs. During the following hours the CK level rose to 99, 600 IU/l and myoglobinuria of 360,000 micrograms/l confirmed the extent of the rhabdomyolysis. The infant was discharged home without detectable sequelae after 2 1/2 weeks. Comparisons with corresponding case reports in the literature lead to the supposition that our patient suffered from a myopathy thus far undiagnosed. To what extent a MH episode may have contributed to the clinical picture cannot be determined at present. The spectrum of adverse reactions to volatile anesthetics and succinylcholine in patients with myopathic disorders is presented and discussed. As in other case reports, the dramatic course described here also demonstrates that in addition to CPR and treatment of the acid-base and electrolyte imbalances, administration of dantrolene should be considered at an early stage.
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PMID:[Cardiac arrest during anesthesia induction with halothane and succinylcholine in an infant. Massive hyperkalemia and rhabdomyolysis in suspected myopathy and/or malignant hyperthermia]. 195 45

Although malignant hyperthermia is still a potentially fatal disease that was marked by a high mortality until recently, lasting damage to the patient can now be prevented by early diagnosis and treatment. The following case demonstrates the special value of capnometry in diagnosing this condition. A 34-year-old man admitted for oral surgery showed symptoms of malignant hyperthermia 5 h after induction of anesthesia. Neuroleptanalgesia had been conducted. The patient had received thiopental and fentanyl for induction of anesthesia and alcuronium and succinylcholine for intubation. The first symptom noticed was an elevation of the end-tidal pCO2 as monitored by capnometry. Additional symptoms, such as a pronounced rise in temperature, blood pressure, and heart rate did not develop until 20-25 min later. The end-tidal oxygen concentration decreased from 30 vol.-% to 26 vol.-%. The patient had to be ventilated with a volume of 25 l/min to keep end-tidal pCO2 under 6 kPa. Treatment with dantrolene was started immediately. Not until 3 h after the onset of the first symptoms did the patient's body temperature and the minute volume needed for ventilation return to normal. Postoperative laboratory findings showed only a slight elevation of creatine kinase and serum lactate. Myoglobin was not detected in serum or urine. This case indicates that capnometry permitted immediate adaptation of controlled ventilation to the patient's increased metabolic rate and early initiation of dantrolene treatment, thus preventing more severe disorders and possible consequences for the patient. Other studies have also suggested the special importance of capnometry. Since the patient refused to give his consent, the diagnosis could not be ascertained by muscle biopsy, and had to be based on symptoms.
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PMID:[Early recognition of malignant hyperthermia using capnometry]. 210 75

In pigs, the serotonin-2 (5-HT2) receptor agonist 1-(2,5-dimethoxy-4-iodophenyl)-2-aminopropane (DOI), 0.8 mg/kg, induced "psychotic" behaviour (e.g., grimacing, backward locomotion, blank stare) and a muscular syndrome, which is known as malignant hyperthermia (MH) in pigs and humans. This syndrome is characterized by generalized skeletal muscle rigidity, leading to an increase in body temperature, marked acidosis, hyperkaliaemia, cyanosis and elevation of lactate, carbon dioxide and the muscle enzyme creatine kinase (CK) in plasma. In pigs which were selectively bred for susceptibility to MH induction by known triggering agents, such as halothane, the administration of DOI was fatal in 3 out of 5 animals. In genetically susceptible pigs, MH was also induced by 5-methoxy-N,N-dimethyltryptamine (5-MeO-DMT), 0.5-1.8 mg/kg, and D-lysergic acid diethylamide (LSD), 60-110 micrograms/kg. Furthermore, 5-MeO-DMT and LSD induced head shakes in the animals, which had not been observed after DOI and could not be blocked by 5-HT2-antagonists, ketanserin (0.5-5 mg/kg) and ritanserin (1-2.5 mg/kg). The psychotomimetic effects of 5-MeO-DMT could be blocked by ketanserin or ritanserin, which, depending on the dose, also reduced or totally prevented the hyperthermia and metabolic changes induced by 5-MeO-DMT in pigs. Administration of 5-MeO-DMT, 1.8 mg/kg, was fatal in 4 of 5 MH-susceptible pigs, whereas pigs injected with this dosage after pretreatment with ketanserin (0.5-5 mg/kg) or ritanserin (1-2.5 mg/kg) did not die. In pigs from MH-resistant littermates, administration of 5-MeO-DMT was not fatal. Comparison of metabolic changes in susceptible and non-susceptible pigs suggested that the marked increase in plasma potassium, which arises principally from damaged muscle cells, is primarily responsible for the fatal effect of DOI and 5-MeO-DMT in genetically susceptible individuals. In MH-susceptible pigs, which were anesthetized, relaxed and artificially ventilated, 5-MeO-DMT did not induce hyperthermia, thus substantiating that the marked hyperthermia observed in conscious pigs was a result of muscle activation and not due to effects on thermoregulation or blood pressure. The results indicate that hallucinogenic drugs with 5-HT2 agonistic effects trigger a life-threatening syndrome, MH, in genetically susceptible pigs. 5-HT2 antagonists, such as ketanserin or ritanserin, are capable of counteracting the fatality of this syndrome.
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PMID:Pharmacodynamic effects of serotonin (5-HT) receptor ligands in pigs: stimulation of 5-HT2 receptors induces malignant hyperthermia. 211 35


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