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

The value of the quantitative electromyogram in the detection of subclinical myopathic signs was tested in a family consisting of 11 members, one of them showing a Malignant Hyperthermia crisis. Furtheron an in vitro contracture test and an ATP-test was performed and the Serum CPK and the isoenzyme in serum and muscle examined. The following conclusions were taken: 1) Although unspecific pattern (in 5 cases more than 12% polyphasic potentials were found, one of them showing a reduction of the mean duration of 30%) the electromyography is a good but not absolutely certain indicator of subclinical myopathic signs in Malignant Hyperthermia. 2) The exclusion of M.H. risk is not possible using a single test. 3) The diagnostic value of not invasive methods, as the electromyography is of special importance. The diagnostic use of many M.H. tests seems necessary.
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PMID:[Electrophysiologic investigations in a family with malignant hyperthermia (author's transl)]. 690 89

A report is made of a 65-year-old male who died of a malignant hyperthermia of 42 degrees C. Symptoms included muscle rigidity at the termination of operation for neck-clipping of an aneurysm of the anterior communicating artery. Latent myopathy was observed in skeletal muscle, and a bleeding focus was present in the left anterior region of the hypothalamus, coinciding with the temperature regulation center. The mechanism of onset of this disease is still poorly understood, but it seems that this disease occurred due to synergic effects of the hypothalamic hemorrhage and the pre-existing myopathy. The serum CPK level at the time of death was abnormally high (250 U), and 3.4% of isozyme CPK1 was detected. The serum myoglobin was 204, 850 ng/ml, a markedly high level, and myoglobinuric nephrosis was present as a result.
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PMID:Malignant hyperthermia. 711 2

Malignant hyperthermia (MH) is a severe familial disease in both the pig and the human, with 70% fatality when fully expressed in humans. MH produces rapid elevation of temperature in response to stresses, of which there are two general kinds: Societal or emotional stress, and chemical stressors. The most commonly encountered stressor is halothane, a general anesthetic in wide use. Besides large temperature increases, there occur some twenty symptoms. Much work in other laboratories has been concentrated on elevated CPK i the plasma. However, all the symptoms are consistent with a single disorder, namely oxidative damage, especially in membranes. A deficiency in the glutathione peroxidase (GPX) system is a prime factor, likely the molecular basis allowing abnormal oxidative damage in the MH pig. Catalase activities are normal in MH pigs, but they have only 20-50% normal GPX activities. The deficiency does not cause oxidative damage. It allows failure or protective mechanisms against it. The nonstressed MH animal exhibits less acute symptoms, e.g. enhanced red cell Heinz bodies, but such animals generally mature. Under stress, their inadequate protective mechanisms dependent on GPX are overwhelmed, resulting in gross symptoms and crisis. It is important to concentrate on the GPX system(s) and their adjacent pentose shunt metabolism. We propose that a deficiency in any of these two systems is the molecular basis of the disease. Many tissues are involve in MH, but the red cell obviously provides a convenient means for assay and for screening. This paper mainly pertains to porcine MH. However, preliminary work with humans indicates that human MH has a similar molecular basis.
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PMID:Malignant hyperthermia (MH): porcine erythrocyte damage from oxidation and glutathione peroxidase deficiency. 729 Nov 94

A case of severe muscular rigidity in a premature male infant born by Caesarean Section under general anaesthesia is described. A probable diagnosis of malignant hyperthermia was supported by the clinical symptoms of muscular rigidity and cyanosis, a creatinine phosphokinase of 24,630 I.U. (Normal 0--100) and a urinary myoglobin of 248 mg/l (normal 6--35). The muscle tone and laboratory values slowly returned to normal over a period of days. Anaesthetic management for a ventriculoperitoneal shunt performed at seven weeks of age included pre and postoperative treatment with dantrolene. No crisis occurred at this time. The parents, who have normal CPK and a negative family history, were advised to treat the child as if he had malignant hyperthermia until such time as a definitive diagnosis can be made.
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PMID:Severe muscular rigidity at birth: malignant hyperthermia syndrome? 737 67

The neuroleptic malignant syndrome (NMS) is a drug-induced disease caused by neuroleptics, but the pathogenesis of NMS is unknown. Since NMS is similar to malignant hyperthermia (MH) in clinical features and treatment, 6 mutations in the skeletal muscle ryanodine receptor (RYR1) gene, which were associated with MH, were investigated in unrelated NMS patients by single-strand conformation polymorphism analysis (SSCP). As a result, MH-susceptible RYR1 mutations were not detected in our NMS patients. A single base substitution, C7278T, was detected in one patient whose serum CPK level was repetitively elevated, but his other major symptoms did not fulfil the clinical criteria for NMS. Our results do not support the association between the neuroleptic malignant syndrome and mutations in the RYR1 gene associated with malignant hyperthermia.
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PMID:No association between the neuroleptic malignant syndrome and mutations in the RYR1 gene associated malignant hyperthermia. 898 16

The preoperative evaluation of the enzymatic level concerning the functioning of the neuromuscular system, is of particular importance. The diseases of these regions may lead to Malignant Hyperthermia (MH) in the course of general anaesthesia. The authors illustrate the case of a young asymptomatic patient with constantly high levels of CPK, who was later affected by Central Core Disease (CCD), a rare hereditary slow progressing myopathy strictly related to MH. The authors suggest tat preoperative CPK levels, though not predictive of a patient to develop MH, can allow identification of patients affected by clinically silent myopathies. This can also acquire legal importance in case of clinical MH manifestation.
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PMID:[Muscular enzyme level disorders and a malignant hyperthermia susceptability test: postoperative study of a patient with elevated CPK]. 965 90

Rabdomyolysis usually occurs after trauma and release of myoglobin from the damaged muscle, i.e.; after ishchemic myopathy due to arterial occlusion or malignant hyperthermia. We encountered a pediatric case of rhabdomyolysis after Ross-Konnos' operation in an 8-yr-old girl with aortic regurgitation. After the first weaning from cardiopulmonary bypass (CPB), ventricular fibrillation occurred due to an insufficiency in coronary blood flow and CPB was resumed with rapid cooling of body temperature. The total CPB lasted for 5 hr 43 min. After the second weaning from CPB, myoglobinuria was found. Furthermore, blisters and abrasions appeared on her back and CPK levels were abnormally elevated (maximum 19,132 IU.l-1) without any elevation of body temperature in the postoperative course. Rhabdomyolysis due to intraoperative hypoperfusion was suspected and diuretics were administrated with a large amount of crystalloid to maintain urine output. The patient showed a good clinical course without acute renal failure. The course of this case suggests that rhabdomyolysis is one of rare complications of CPB and an early correct diagnosis of rhabdomyolysis and forced diuresis at an early stage are important to avoid acute renal failure.
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PMID:[A case of rhabdomyolysis after open heart surgery in a child]. 1121 44

A 57-year-old man with lung tumor was scheduled for right middle lobectomy under general anesthesia. The patient received glycerin enema 2 hours before anesthesia. Anesthesia was induced with propofol, fentanyl, ketamine and vecuronium. After the induction, urine of dark-red color was drained through the urinary catheter. Massive (3+) occult blood and few erythrocytes in the urine sediment were observed. Furthermore, blood analysis showed hemolysis with mild renal dysfunction (Cr 1.3 mg x dl(-1)). Although serum CPK and myoglobin increased, there was no apparent symptom that supported the onset of rhabdomyolysis induced by anesthetics, acute myocardial infarction or malignant hyperthermia. At this time, we noticed that blood sample taken before the induction had been hemolysed. With all the above information in mind, we suspected that the main cause of the hemoglobinuria could be the enema and the surgery was canceled. The patient made a good progress with laboratory data normalized on the 4th postanesthesia day. However, rectal ulcer developed as a possible late complication of the enema. Although it is well-known that glycerin enema could cause hemolysis, renal failure and rectal ulcer, the increase of CPK and myoglobin in serum made the diagnosis difficult from other conditions leading to rhabdomyolysis in this case.
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PMID:[Case of hemoglobinuria following glycerin enema]. 1757 10

The condition of pale, soft, exudative (PSE) pork has long been considered to be mainly a post mortem phenomenon. There is now substantial evidence that such pigs are suffering from a kind of myopathy, which predisposes them to an abnormal post mortem metabolism. Genetic studies on PSE muscle indicate a moderate heritability for various post mortem muscle quality traits. Reliable methods for determining the abnormal condition in the live animal would make it possible to select more effectively and economically against stress and PSE-susceptibility. Three possible methods are: (1) Analysis of blood serum for CPK, aldolase, GOT or other enzyme activities with and without preceding exercise; and for blood groups. (2) Muscle biopsy analysis for glucose-6-phosphate, lactate or energy-rich phosphates. (3) Non-destructive testing of young pigs for sensitivity to the Malignant Hyperthermia Syndrome by allowing them to inhale the anaesthetic halothane (fluothane) for a 5 min period. The development of muscular rigidity and stiffness indicates a susceptibility to stress and a potential for PSE meat. The relationships of the various methods to ultimate muscle and carcass quality, as well as the problems inherent to each method, are discussed. It is concluded that the third test seems to be the most promising for application in the breeding of pigs for optimal stress resistance and muscle quality.
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PMID:Methods of predicting pale, soft, exudative pork and their application in breeding programmes-A review. 2205 69

Malignant hyperthermia is a hypermetabolic syndrome that appears in susceptible patients after exposure to certain anaesthetic drugs (succinylcholine, inhalation anaesthetics). Its incidence in Spain is 1 in 40,000 adults, with a 10% mortality rate. It is induced by an abnormal regulation of the ryanodine receptors, producing a massive release of calcium from the sarcoplasmic reticulum in the striate muscle. Clinical manifestations include: CO2 increase, tachycardia, haemodynamic instability, metabolic and respiratory acidosis, profuse sweating, hyperpyrexia, CPK increase, myoglobinuria, kidney failure, disseminated intravascular coagulation (DIC), and ending in cardiac arrest. Dantrolene sodium is a ryanodine receptor antagonist, and inhibits the release of intracellular calcium. Definitive diagnosis is achieved by the exposure of muscle fibres to caffeine and halothane. Protocols can help guarantee a reliable and secure management when this severe event occurs.
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PMID:Clinical protocol for the management of malignant hyperthermia. 2763 84


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