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

Malignant hyperthermia is a skeletal muscle disorder thought to be genetically acquired. Inhalation anesthesia presents a dangerous risk to the patient predisposed to the condition.
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PMID:Outpatient dental treatment of pediatric patients with malignant hyperthermia: report of three cases. 153 44

This review examines the nature of masseter spasm, its controversial relation to malignant hyperpyrexia, and the management of the child who develops it during induction of anaesthesia.
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PMID:Masseter spasm after suxamethonium in children. 154 61

Schwartz-Jampel syndrome is a rare autosomal recessive disorder. Joint contractures, generalised myotonia, skeletal anomalies, and facial dysmorphism are common features; malignant hyperthermia is a potentially lethal complication during anaesthesia.
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PMID:Schwartz-Jampel syndrome (chondrodystrophic myotonia). 155 48

Malignant hyperthermia (MH) is a rare genetic myopathy that was first described as a fatal complication of general anesthesia in 1960. It is estimated to affect approximately 1 in 15,000 pediatric patients and 1 in 40,000 adult middle-aged patients. The mode of transmission is genetic: the severest form is autosomal dominant, and the less severe, autosomal recessive. Thus, both men and women can have MH, although there is a slightly higher incidence in the male pediatric population. Malignant hyperthermia is usually triggered by halogenated anesthetic agents with or without depolarizing muscle relaxants. The classic diagnostic triad consists of skeletal muscle rigidity, metabolic acidosis, and elevated body temperature. The definitive diagnosis is suspected susceptible individuals is revealed by exposing an intact muscle fiber to caffeine and halothane in varying concentrations. An abnormal contracture response is hypothesized to be the result of an increase in the release of calcium ion from the sarcoplasmic reticulum in response to neuronal stimulation leading to a hypermetabolic state. The mainstay of treatment is dantrolene, given either prophylactically in susceptible patients or immediately whenever a malignant hyperthermic episode is suspected.
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PMID:Malignant hyperthermia: a review. 156 Feb 93

Operative charts were reviewed in 86 patients with Charcot-Marie-Tooth disease, a condition characterized by chronic muscular denervation. A total of 161 surgical procedures was performed. Major complications were few, and only one operative death occurred, unrelated to anaesthesia. Succinylcholine and malignant hyperthermia triggering agents were used in 41 (48%) and 77 (90%) patients, respectively, without untoward effects. Contrary to previous reports, this survey supports the safe use of succinylcholine and MH triggering agents in this disease.
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PMID:Anaesthesia for Charcot-Marie-Tooth disease: a review of 86 cases. 156 65

Malignant hyperthermia (MH) is a genetic disease characterized by hypermetabolism in skeletal muscle following a triggering stimulus and can be reversed or pretreated with dantrolene sodium. The myoplasmic free [Ca2+] was measured, using Ca2+ selective microelectrodes in vivo in the superficial fibers of the sartorius muscle of eight MH-susceptible and eight control subjects. Both groups received continuous epidural anesthesia with chloroprocaine 3%. In both the control and MH muscle fibers, the myoplasmic free [Ca2+] was measured before and after the intravenous administration of a cumulative dantrolene dose of 0.5, 1.5, and 2.5 mg/kg. The mean resting myoplasmic free [Ca2+] was 0.112 +/- 0.004 microM (mean +/- SEM n = 32) in the control and 0.485 +/- 0.022 microM (n = 33) in the MH subjects. In the MH subjects, dantrolene induced a dose-dependent reduction in myoplasmic free [Ca2+]. The 0.5-mg/kg dose reduced it to 0.326 +/- 0.017 microM (n = 22), the 1.5-mg/kg dose to 0.233 +/- 0.015 microM (n = 25), and the 2.5-mg/kg dose to 0.092 +/- 0.008 microM (n = 26). In controls, dantrolene also reduced resting myoplasmic free [Ca2+] but to a lesser extent. The 0.5-mg/kg dose reduced it to 0.096 +/- 0.004 microM (n = 22), the 1.5-mg/kg dose to 0.077 +/- 0.003 microM (n = 23), and the 2.5-mg/kg dose to 0.068 +/- 0.002 microM (n = 27). The results of the study extend our previous findings in humans and swine and demonstrate that it is possible to measure myoplasmic free [Ca2+] in vivo in humans.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Effects of dantrolene on myoplasmic free [Ca2+] measured in vivo in patients susceptible to malignant hyperthermia. 157 38

Etomidate is a useful addition to the list of available anesthetic agents. When faced with an animal with cardiovascular instability, cirrhosis, an intracranial lesion, susceptibility to malignant hyperthermia, anaphylactoid tendencies, or one that requires cesarean section, one should consider using etomidate. In addition, it provides a safe method for total intravenous anesthesia in situations in which the nature of the surgery precludes the use of an endotracheal tube, when the use of an inhalant is undesirable for any reason, or when inhalant anesthetic equipment is unavailable.
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PMID:Advantages of etomidate use as an anesthetic agent. 158 53

In humans genetically predisposed to malignant hyperthermia, anesthesia can induce skeletal muscle rigidity, hypermetabolism, and high fever, which, if not immediately reversed, can lead to tissue damage or death. The corresponding condition in swine leads to stress-induced deaths and devalued meat products. Abnormalities in the Ca2+ release channel of skeletal muscle sarcoplasmic reticulum (the ryanodine receptor) have been implicated in the cause of both the porcine and human syndromes by physiological and biochemical studies and genetic linkage analysis. In swine, a single founder mutation in the ryanodine receptor gene (RYR1) can account for all cases of malignant hyperthermia in all breeds, but a series of different RYR1 mutations are likely to be uncovered in human families with MH. Moreover, lack of linkage between malignant hyperthermia and RYR1 in some families indicates a heterogeneous genetic basis for the human syndrome.
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PMID:Malignant hyperthermia. 158 59

It has been claimed that the combination of halothane and succinylcholine, commonly used for anesthetic induction during short pediatric otolaryngologic procedures, is associated with a 1% incidence of masseter spasm (MS) which may be an early sign of malignant hyperthermia (MH). An 18-month retrospective chart review of all patients undergoing general anesthesia at the Children's Hospital of Pittsburgh (n = 14, 112) was conducted to assess the incidence of MS and its management. In addition, a separate subgroup of patients identified as being at risk for MH was also evaluated. In the otolaryngology service, the incidence of developing MS was 2 of 206 (1%) in children who were anesthetized with halothane and received succinylcholine, patients were identified in the MH high-risk group, and none developed MH. The findings affirmed the risks of using this combination of anesthetic and neuromuscular blocking agents during induction and the need for establishing management guidelines.
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PMID:Masseter spasm and malignant hyperthermia: a retrospective review of a hospital-based pediatric otolaryngology practice. 159 51

In this study we investigated in vitro and in vivo effects of propofol in malignant hyperthermia susceptible (MHS) patients in order to assess the safety of propofol infusion as a non-triggering anaesthetic technique for diagnostic and therapeutic procedures. In vitro, human MHS muscle samples were exposed to propofol and changes in (a) baseline tension and (b) contracture tension on exposure to halothane and caffeine were measured. In vivo, (a) anaesthesia was induced in ten muscle biopsy positive MHS patients with propofol 2.5 mg/kg and (b) anaesthesia was produced in five muscle biopsy positive MHS patients with infusions of propofol up to 10 mg/kg/hr. In vitro, human MHS muscle did not develop contractures with propofol alone. Propofol had no significant effect on contracture development in response to halothane and caffeine. In vivo, no evidence of an MH response was detected following induction or maintenance of anaesthesia with propofol. Our results and literature review are in agreement that propofol is a 'safe' induction and maintenance agent in MHS patients. Propofol can be used for muscle biopsy anaesthesia because it does not alter the sensitivity of diagnostic muscle biopsy testing.
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PMID:Propofol is a 'safe' anaesthetic agent in malignant hyperthermia susceptible patients. 159 50


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