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)

A 53-year-old woman was scheduled for elective surgery of a L4-L5 lumbar disc prolapse. Preoperatively, she reported of a hereditary muscle disease in her family which could be identified as familial hyperkalaemic periodic paralysis. In patients with familial periodic paralysis, only limited information is available in the current anaesthesiological literature. This is especially true of the hyperkalaemic form which was separated from the hypokalaemic form in 1957. Most patients suffering from periodic paralysis will develop myotonic symptoms in time, but evidence is lacking that the incidence of malignant hyperthermia (MH) is higher than in normals. However, abnormalities of the electrocardiogram (ECG) due to changes in the potassium serum levels are not unusual, and the anaesthesiologist must be aware of cardiac arrhythmias. In the present case, the patient was anaesthetized using fentanyl, midazolam, and vecuronium, and ventilated with 66% nitrous oxide in oxygen. Radial artery blood pressure, end-tidal CO2, and nasopharyngeal temperature were monitored continuously. Surgery was completed after 2 hours with no abnormalities in the intraoperative course. Recovery from anaesthesia was uneventful. During the postoperative follow-up for 1 week, further attacks of paralysis, intermittent sodium-potassium imbalance of the muscle cell membrane appears to be the primary pathogenetic factor. In this special disease, prevention of carbohydrate depletion and the avoidance of muscle relaxants are recommended in the anaesthesiological literature. However, a specified regimen of general anaesthesia has not yet been outlined. In our special case, the use of anaesthetics deemed to be safe in MH susceptible patients produced an uneventful perioperative course.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Anesthesia in familial hyperkalemic periodic paralysis]. 832 4

A 6-year old female child received succinylcholine (1 mg.kg-1) and isoflurane (concentrations of 1.5-2 percent) and developed at the end of surgery a hypermetabolic syndrome suggestive of malignant hyperthermia (MH) with masseter muscle spasm, muscle rigidity, tachypnea, systolic hypertension (140 mm Hg), tachycardia (205 beats.min-1), hypercarbia (end expiratory CO2 71 mmHg), and an increase in body temperature (39.2 degrees C). The child responded well to therapy which included cooling, hyperventilation with pure oxygen and dantrolene administration. However, blood creatine kinase and myoglobin elevations were moderate (respectively 375 IU.L-1 and 114 micrograms.L-1) and an in vitro halothane and caffeine contracture test was negative. Differential diagnostic proposals are discussed and compared to the clinical incident.
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PMID:Malignant hyperthermia suggestive hypermetabolic syndrome at emergence from anesthesia. 871 51

The authors report on a course of malignant hyperthermia (MH) in an almost 5-years old boy. In the past, he had been anaesthetized two times with halothane without complications. The causative triggering agent was sevoflurane, a new user-friendly substance for paediatric anaesthesia. Forty five minutes after induction of anaesthesia he developed symptoms of a MH-crisis with increase in endexspiratory CO2 up 87 mmHg and followed by an increase in heart rate up to 160 beats/minute. The blood gas analysis showed a respiratory and metabolic acidosis. The timely administration of dantrolene rapidly reversed the life-threatening signs and prevent progression of the disease. It is apparent that monitoring of endtidal carbon dioxide by means of capnometry is of crucial importance in detecting MH at an early stage, and appropriate treatment is being instituted more promptly. By such early recognition, and treatment with dantrolene, we can reasonably except a further decrease in mortality and morbidity of this enigmatic disorder.
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PMID:[Malignant hyperthermia and sevoflurane--a case report]. 944 66

Malignant hyperthermia is a rare autosomal dominant trait that predisposes affected individuals to great danger when exposed to certain anaesthetic triggering agents (such as potent volatile anaesthetics and succinylcholine). A sudden hypermetabolic reaction in skeletal muscle leading to hyperthermia and massive rhabdomyolysis can occur. The ultimate treatment is dantrolene sodium a nonspecific muscle relaxant. Certain precautions should be taken before anaesthesia of patients known to be susceptible to malignant hyperthermia. These include the prohibition of the use of triggering agents, monitoring of central body temperature and expired CO2, and immediate availability of dantrolene. In addition, careful cleansing of the anaesthesia machine of vapours of halogenated agents is recommended. If these measures are taken, the chances of an MH episode are greatly reduced. When malignant hyperthermia-does occur in the operating room, prompt recognition and treatment usually prevent a potentially fatal outcome. The most reliable test to establish susceptibility to malignant hyperthermia is currently the in vitro caffeine-halothane contracture test. It is hoped that in the future a genetic test will be available.
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PMID:Malignant hyperthermia. 953 80

The intra-operative differential diagnosis between thyroid crisis and malignant hyperthermia can be difficult. Also stress alone can trigger MH. The purposes of this study were: 1) to investigate the metabolic and hemodynamic differences between thyroid crisis and MH, 2) determine how thyroid crisis affects the development of MH, and 3) determine if the stress of thyroid crisis can trigger MH in susceptible individuals. We studied MH susceptible and normal swine. Two groups of animals (MH susceptible and normal) were induced into thyroid crisis (critical core hyperthermia, sustained tachycardia and increase in oxygen consumption) by pretreatment with intraperitoneal triiodothyronine (T3) followed by large hourly intravenous injections of T3. Two similar groups were given intravenous T3 but no pretreatment. These animals did not develop thyroid crisis and served as controls. Thyroid crisis did not result in metabolic changes or rigidity characteristic of an acute episode of MH. When the animals were subsequently challenged with MH triggering agents (halothane plus succinylcholine) dramatic manifestations of fulminant MH episodes (acute serious elevation in exhaled carbon dioxide, arterial CO2, rigidity and acidemia) were noted only in the MH susceptible animals. Although thyroid crisis did not trigger MH in the susceptible animals it did decrease the time to trigger MH (14.1 +/- 7.2 minutes versus 47.2 +/- 17.7 minutes, p < 0.01) in susceptible animals. Hormone induced elevations in temperature and possibly other unidentified factors during thyroid crisis may facilitate the triggering of MH following halothane and succinylcholine challenge.
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PMID:Differential diagnosis of thyroid crisis and malignant hyperthermia in an anesthetized porcine model. 1009 96

We report the case of a 31-year-old man with Graves' disease who manifested malignant hyperthermia during subtotal thyroidectomy. His past medical history and family history were unremarkable. Before surgery, his condition was well controlled with propylthiouracil, beta-adrenergic blocker and iodine. During the operation, anesthesia was induced by intravenous injection of vecuronium and thiopental, followed by suxamethonium for endotracheal intubation. Anesthesia was maintained with nitrous oxide and sevoflurane. One hour after induction of anesthesia, his end tidal carbon dioxide concentration (ET(CO2)) increased from 40 to 50 mmHg, heart rate increased from 90 to 100 beats per min and body temperature began to rise at a rate of 0.3 degrees C per 15 min. Suspecting thyroid storm, propranolol 0.4 mg and methylprednisolone 1,500 mg were administered, which, however, had little effect. Despite the lack of muscular rigidity, the diagnosis of malignant hyperthermia was made based on respiratory acidosis. Sevoflurane was discontinued and dantrolene was given by intravenous bolus. Soon after the treatment, ET(CO2), heart rate and body temperature started to fall to normal levels. His laboratory findings showed abnormally elevated serum creatine phosphokinase and myoglobin but normal thyroid hormone levels. Since dantrolene is efficacious in thyrotoxic crisis and malignant hyperthermia, an immediate intravenous administration of dantrolene should be considered when a hypermetabolic state occurs during anesthesia in surgical treatment for a patient with Graves' disease.
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PMID:Malignant hyperthermia in a patient with Graves' disease during subtotal thyroidectomy. 1145 72

Malignant hyperthermia is an autosomal-dominant inherited disorder of the skeletal muscle cell characterized by a hypermetabolic response to all commonly used inhalational anaesthetics and depolarizing muscle relaxants. The clinical syndrome includes muscle rigidity, hypercapnia, tachycardia and myoglobinuria as result of increased carbon dioxide production, oxygen consumption and muscle membrane breakdown. In human beings and animals susceptible to malignant hyperthermia, it is generally accepted that an increase in the level of myoplasmic free calcium is the cause of the syndrome. Various hypotheses have been proposed to account for the increase of intracellular calcium levels, e.g. a defect in the calcium release channel of the sarcoplasmic reticulum (ryanodine receptor), an abnormality of the excitation-contraction coupling mechanisms, or alterations in second messenger systems of skeletal muscles. This review gives an overview of the main features of this disease and recent advances in research including pathophysiology, treatment, diagnosis and genetics as well as association with other disorders.
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PMID:Malignant hyperthermia. 1155 40

Susceptibility to malignant hyperthermia is diagnosed by contracture test, which requires an open muscle biopsy sample. We postulated that intramuscular injection of caffeine increases local carbon dioxide pressure (pCO(2)) in individuals susceptible to malignant hyperthermia but not in those who are non-susceptible or in healthy individuals. We measured pCO(2) in the rectus femoris muscle during local stimulation with 500 microL caffeine 80 mmol/L in 12 patients susceptible to malignant hyperthermia, eight non-susceptible individuals, and seven healthy controls. In susceptible individuals, pCO(2) temporarily increased to a maximum of 63 mm Hg (IQR 61-69) compared with 44 mm Hg (43-47) in non-susceptible participants (p=0.003) and 42 mm Hg (41-46) in controls (p=0.003). There were no systemic side-effects. Metabolic monitoring of the highly diffusible CO(2) stimulated by caffeine might allow a minimally invasive test for susceptibility to malignant hyperthermia.
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PMID:Diagnosis of susceptibility to malignant hyperthermia by use of a metabolic test. 1451 45

A 12-year-old boy with Duchenne muscular dystrophy underwent posterior spinal fusion for progressive scoliosis. Preoperative evaluation was focused on respiratory function as well as cardiac function, which revealed markedly reduced respiratory reserve (FVC 0.77 l, %FVC 25.9%, FEV1.0 0.48 l, %FEV1.0 62%) and well-preserved biventricular function. A possible association between malignant hyperthermia and Duchenne muscular dystrophy has been documented. Thus anesthesia was administered without triggering agents. Propofol and fentanyl were used for induction and maintenance of anesthesia, and the patient was ventilated with O2-nitrous oxide mixture. The anesthesia machine, prepared by using a disposable circuit and fresh CO2-absorbent and disconnecting the vaporizers, was flushed with O2 at a rate of 10 l.min-1 for 20 minutes before use. A small dose of vecuronium was administered while monitoring the train-of-four ratio. The bispectral index (BIS) was utilized to optimize the depth of anesthesia so that the wake-up test could be performed promptly on surgeon's request while avoiding the intraoperative awareness. The BIS was helpful in continuously assessing the wakening process. BIS increased from 40's to 80's in 15 minutes after discontinuation of propofol and nitrous oxide during the test. The patient was kept under close observation postoperatively without any sign of malignant hyperthermia.
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PMID:[Application of bispectral index (BIS) monitoring to anesthetic management of posterior spinal fusion in a patient with Duchenne muscular dystrophy]. 1216 84

Early clinical signs, triggering agents, time to onset of reaction, mortality and methods of treatment were identified in 123 suspected malignant hyperthermia reactions. In vitro contracture test results were compared with clinical signs and the Malignant Hyperthermia Clinical Grading Scale. Increased end-tidal carbon dioxide is the earliest sign when not preceded by masseter spasm. Earlier diagnosis reduces the incidence of rigidity and severe metabolic acidosis. The combination of suxamethonium and a potent volatile anaesthetic agent triggers an earlier reaction compared with a volatile agent alone. There has been zero mortality since 1981, essentially due to a combination of advanced monitoring capability, increased anaesthetist awareness of malignant hyperthermia, and dantrolene availability. DNA analysis has identified nine New Zealand families with ryanodine receptor gene mutations. A positive DNA test indicates malignant hyperthermia susceptibility with "causative" mutations but discordance requires that negative DNA tests are confirmed with in vitro contracture test. This test also demonstrated the shortcomings of the Malignant Hyperthermia Clinical Grading Scale.
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PMID:Suspected malignant hyperthermia reactions in New Zealand. 1263 9


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