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)

We evaluated six boys who had developed isolated masseter muscle spasm following intravenous succinylcholine. All were receiving halothane by inhalation.l In vitro muscle contracture tests utilizing halothane and caffeine were performed. Four of the six boys had contracture response similar to those of malignant hyperthermia susceptible patients. Rigidity following succinylcholine should prompt the clinician to consider malignant hyperthermia but has been associated with other myopathic conditions as discussed.
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PMID:Masseter spasm induced by succinylcholine in children: contracture testing for malignant hyperthermia: report of six cases. 705 43

We present a 17-year-old boy with Becker muscular dystrophy (BMD) who developed hyperthermia and heart failure after general anesthesia. He presented clinical features of malignant hyperthermia (MH), and had masseter spasm and elevated body temperature (38.7 degrees C) with very high serum CK activity (107,000 IUl-1). Dystrophin tests confirmed a clinical diagnosis of BMD in the patient, i.e. faint and patchy immunostaining pattern of skeletal muscle, truncated dystrophin protein and a deletion of exons 3 and 4 of the dystrophin gene. To inquire into the mechanism of MH associated in the patient, we tested caffeine contracture reaction by the skinned fiber method. We found an increased sensitivity to caffeine only in type 1 muscle fibers. The rate of Ca(2+)-induced Ca2+ release (CICR) was normal, suggesting that the mechanism of "MH" observed in our patient with BMD is not the same as that of classical MH. A possible mechanism might be related to derangements of the sarcoplasmic reticulum membrane in BMD, which sensitize the membrane to caffeine or other agents.
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PMID:Malignant hyperthermia in a patient with Becker muscular dystrophy: dystrophin analysis and caffeine contracture study. 771 42

During palatoplasty on a 9-year-old girl with no personal or familial history of malignant hyperthermia, the temperature monitor reported an increase in temperature. Additionally, the surgeon thought the patient's jaw muscle was in spasm. While preparations were made for treatment of malignant hyperthermia, the temperature probe was tested and found to be defective. When it was replaced, the patient's temperature was within normal range. When the temperature probe was tested 6 days later, it was working properly. The cause of the problem may have been moisture in the connection between the probe and the exterior cable, which eventually evaporated. Decision algorithms can assist in such situations to distinguish between a medical problem and a mechanical problem with the monitor.
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PMID:Assessment of masseter spasm complicated by a faulty temperature probe. 788 May 18

The purpose of this study was to gain new insights in the role of succinylcholine in the initiation of malignant hyperthermia (MH). The intravenous (i.v.) administration of succinylcholine (2.0 mg/kg) induced fasciculations and masseter spasm in both normal swine and those susceptible to MH. However, the amplitudes and durations of generalized fasciculations were significantly greater in the susceptible animals that subsequently developed a fulminant episode of MH: succinylcholine induced not only tachycardia, hyperthermia, contractures, and increases in PaCO2 and lactate, all classic indicators of an episode, but also an initial severe hypotension. The mean arterial pressure in these swine decreased from 115 +/- 6 mm Hg to 60 +/- 12 mm Hg (mean +/- SD), 1 min after the administration of succinylcholine. Normal swine developed neither cardiovascular effects nor altered metabolism in response to succinylcholine. The pretreatment of animals with a nondepolarizing muscle relaxant (pancuronium 0.1 mg/kg) minimized fasciculations induced by succinylcholine, but did not prevent the hypotension nor episodes of MH in the susceptible swine. In the pretreated and untreated susceptible swine, dantrolene was an equally effective treatment. Plasma catecholamine levels after succinylcholine administration were increased only in the susceptible swine without the pancuronium pretreatment. We concluded that the effects of succinylcholine on skeletal muscle and/or on other tissues play a significant role in the initiation of a MH episode in swine with this genetic disorder, and that these effects are not dependent on an abnormal sensitivity for succinylcholine-induced skeletal muscle fasciculations in these animals.
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PMID:Response to succinylcholine in porcine malignant hyperthermia. 801 Apr 26

A case of malignant hyperpyrexia (MH) occurring in a patient undergoing orthognathic surgery is reported which resulted in the procedure being abandoned prior to completion. The oral surgeon's role in recognising the early clinical signs of masseteric spasm and central cyanosis is highlighted. The case shows that with proper investigation and management the MH susceptible patient was able to have a second anaesthetic with safety to complete the surgery.
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PMID:Malignant hyperpyrexia in oral surgery--case report and literature review. 806 95

Malignant hyperthermia, a inheritant metabolic disease can be induced by some anesthetic agents. The morbid conditions and clinical manifestations in two cases of malignant hyperthermia were studied. The two basic conditions are necessary in the occurrence of malignant hyperthermia: familial inheritant defects, i.e. abnormal membranes that encase myosarkoplasm; and contact of anesthetic agents. The main clinical manifestations of malignant hyperthermia include muscular tetanic spasm and abrupt rising of body temperature. Five aspects of early prognosis and prompt treatment are given.
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PMID:[Malignant hyperthermia: preoperative prognosis, prevention and treatment]. 811 62

At present the in vitro caffeine-halothane contracture test is the only test to predict susceptibility to malignant hyperthermia (MH) with acceptable sensitivity and specificity. Anaesthesia is necessary for the excision of muscle bundles from the vastus lateralis muscle. MATERIAL AND METHODS. Between 1983 and 1991, muscle biopsies were taken from 350 patients, 233 children and 117 adults. In 197 cases, trigger-free general anaesthetics were supplemented by ventilation, via mask in 124 cases and via tracheal intubation in 73 cases. One hundred and fifty-three muscle biopsies were performed under regional or local anaesthesia with or without sedation. Fourteen of the patients with regional anaesthesia needed an additional general anaesthetic to tolerate the operation. For premedication midazolam was administered orally. The general anaesthetics consisted of etomidate or propofol with fentanyl or alfentanil, always with nitrous oxide and oxygen. For local anaesthesia we used procaine or prilocaine. During the first 3 years local infiltration of the thigh was used, but subsequently direct nerve blockade of the femoral nerve and the lateral cutaneous femoral nerve was preferred. RESULTS. In all cases of general anaesthesia and in 91.6% of cases of regional anaesthesia operating conditions were very good. Fourteen (8.4%) of the patients with regional anaesthesia needed an additional general anaesthetic. There were no severe complications noticed. Moderate complications were found in 29 of the 194 paediatric general anaesthesias (15%) and in 5 of the 117 regional anaesthesias in adults (4%); all were easy to treat. The 50 paediatric regional anaesthesias and the three general anaesthesias in adults were without complications. DISCUSSION. For muscle biopsies, trigger-free general anaesthesia can be recommended as well as peripheral nerve blockades. Complete monitoring is necessary, even for this minor procedure: ECG, blood pressure, pulse oximetry, capnometry, measurement of body temperature and blood gas status. Differential diagnosis in negative test results. In patients who suffer an anaesthetic incident, the following disease must be considered: myopathies (especially the congenital myopathies and muscular dystrophies), respiratory problems due to pulmonary infection and obstruction, metabolic disorders of various origins, and the problem of masseter spasm.
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PMID:[Diagnosis of malignant hyperthermia susceptibility. 2. Anesthesia for muscle biopsy. Differential diagnosis in negative test results]. 825 Feb 2

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

A 13-year-old boy presenting for correction of bat ears was anaesthetised with thiopentone and suxamethonium, the administration of which was followed by jaw spasm, poor peripheral perfusion (without cyanosis) and marked tachycardia. The procedure was abandoned, dantrolene and Ringer lactate IL were given intravenously and the patient regained consciousness 1 h later. Levels of serum myoglobin, urinary myoglobin and creatine kinase were followed until they returned to normal. Despite a peak serum myoglobin of 58.000 micrograms.l-1 and peak urinary level of 446,000 micrograms.l-1, no renal impairment occurred. Subsequent testing for susceptibility to malignant hyperthermia proved positive for the patient and four other members of the family.
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PMID:Serum and urinary myoglobin following an aborted malignant hyperthermia reaction. 898 72

The mitochondrial myopathies are a rare group of conditions affecting the respiratory chain and oxidative phosphorylation. The anesthetic management of a 6-year-old girl with complex I respiratory chain deficiency requiring surgery for a fractured hip is presented and discussed. Potential problems were masseter spasm, tendency to develop lactate acidosis, and malignant hyperthermia susceptibility. These problems were avoided by the use of a laryngeal mask airway, allowing the patient to spontaneously ventilate; caudal analgesia; and maintenance of anesthesia with a proprofol infusion.
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PMID:Anesthesia for a child with complex I respiratory chain enzyme deficiency. 979 22


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