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)

General anesthesia permits surgery involving large operative fields without limit to time. It is essential in children and uncooperative adults and provides an immobile, quiet patient who does not recall the operative procedure. Local and regional anesthesia re indicated when the surgical field is limited in size and extent. Neuroleptanesthesia in ophthalmology usually involves the administration of droperidol followed by fentanyl followed in intubation, sometimes using a muscle relaxant, and maintenance of anesthesia with nitrous oxide. The terrifying dreams produced by ketamine may be minimized by preoperative administration of diazepam and a quiet recovery period. Attempts to eliminate the oculocardiac reflex are unpredictable and unreliable and, with careful monitoring of anesthesia, elimination does not seem essential. Malignant hyperthermia is an autosomal dominant disorder occurring mainly with the administration of halogenated fluoroethane and depolarizing muscle relaxants. It is often signaled by sudden and prolonged muscle spasm following the injection of the muscle relaxant. Treatment must be directed toward hyperventilation using oxygen, correction of the metabolic respiratory acidosis, and reduction of fever.
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PMID:Current trends in ophthalmic anesthesia. The Second Walter S. Atkinson Lecture. 115 56

A 32-year-old man survived malignant hyperthermia which developed in conjunction with laparotomy. Muscles biopsy indicated acute necrosis of muscle fibers as well as changes consistent with a latent myopathy. Clinical, laboratory and morphological studies of the relatives revealed familial subclinical myopathy. The hyperthermia resulting from the anaesthesia is thought to be casually related to the myopathy, and members of the family have been warned against this potential risk.
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PMID:[Clinical and morphological findings in a case of malignant hyperthermia (author's transl)]. 115 6

Malignant hyperthermia syndrome associated with various anesthetic agents has been well documented, but none has been reported with enflurane (Ethrane). This particular case is the first involving exposure for the second time to enflurane anesthesia after a six-month interval in which malignant hyperthermia resulted. A patient subject to malignant hyperthermia does not necessarily develop rigidity after succinylcholine for the intubation as noticed in this case. However, with the abnormal production of heat in the soda lime canister with tachycardia, tachypnea, rapidly rising body temperature and hypertonicity of skeletal muscles during anesthesia one should suspect malignant hyperthermia. The treatment of malignant hyperthermia involves cooling procedures, correction of acidosis, use of steroids and procainamide, and acetaminophen to facilitate lowering the body temperature.
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PMID:Malignant hyperthermia associated with enflurane anesthesia: a case report. 116 60

Malignant hyperpyrexia is a highly lethal complication of general anesthesia. It occurs with sufficient frequency, particularly in children and young adults, to justify use of continuous temperature monitoring during adminnstration of general anesthesia. Fever may be a late manifestation of the malignant hyperpyrexia syndrome, and it is important to be aware of the early signs. Prompt recognition and aggressive management should lower the high mortality known to occur with this complication. Family members should be screened for CPK elevations to determine possible susceptibility to malignant hyperpyrexia. Two case reports illustrate these points of diagnosis and management.
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PMID:Malignant hyperpyrexia during anesthesia in childhood. 126 56

The effectiveness of intravenous administration of dantrolene in prevention and treatment of fulminant malignant hyperthermia (MH) initiated by halothene and succinylcholine (SCh) in genetically susceptible swine was assessed. In six animals, prior administration of dantrolene in doses of 5 mg/kg or more prevented MH, while 1 or 3 mg/kg attenuated MH, and 0.1 mg/kg had no effect. In ten additional swine, therapy was not started until MH was fulminant. Five of these were then given supportive therapy only (discontinuation of anesthesia, hyperventilation with oxygen, surface cooling, and NaHCO3). The remaining five received the same supportive therapy, plus dantrolene (7.5 mg/kg). With supportive therapy only, arterial blood pH, Po2 and Pco2 returned toward normal, but oxygen consumption (Vo2), blood lactate, potassium (K+), catecholamines, and temperature continued to increase and the course of MH was unaltered. When dantrolene was added to supportive therapy, Vo2, lactate, K+, catecholamines, and temperature decreased, and the course of MH was dramatically slowed and, apparently, reversed.
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PMID:Dantrolene in porcine malignant hyperthermia. 127 16

Effort rhabdomyolysis is a syndrome which takes the form of a pathology with different etiopathogenetic stages. General anesthesia may trigger off acute muscular cytolysis which is probably influenced by the inflammatory surgical pathology itself. The observation of two cases of post-anesthetic rhabdomyolysis following muscular stress suggest that the exhaustion of muscle energy reserves and the consequent alteration of the ATP/ADP ratio may act as a trigger mechanism for this syndrome. Its close relationship with malignant hyperthermia makes a precise anamnesis vitally important, especially in relation to recent intense physical effort in patients undergoing emergency surgery for acute pathologies.
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PMID:[Post-anesthesia rhabdomyolysis syndrome following muscular stress. 2 case reports]. 129 23

Malignant hyperthermia (MH) is a life threatening complication following anaesthesia with potent inhalational agents and suxamethonium. The signs of MH are caused by increased metabolism and secondary stimulation of the sympathetic nervous system due to uncontrolled, high intracellular concentrations of calcium in skeletal muscle. The hyperthermia is secondary to the increased energy turnover. Calcium release from the sarcoplasmic reticulum is increased due to a low threshold for release and prolonged opening of the calcium channel upon normal stimulation. The gene encoding the calcium channel is localized on chromosome 19 in humans, and a substitution of cysteine for arginine has recently been described in one family with MH.
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PMID:[Malignant hyperthermia]. 131 Mar 56

Anaesthesia can induce skeletal muscle rigidity, hypermetabolism and high fever in humans genetically predisposed to malignant hyperthermia. If not immediately reversed, such episodes can lead to tissue damage and death. In swine with the corresponding condition, stress can induce death or lead to devalued meat products. Since muscle contraction is controlled by sarcoplasmic Ca2+, the abnormality, as reviewed here by David H. MacLennan, could reside in the skeletal muscle Ca(2+)-release channel gene, RYR1. Several observations support the view that a single RYR1 mutation is causal of malignant hyperthermia in all breeds of pigs and in at least some human families: the substitution of Cys for Arg615 as the sole deduced amino acid sequence change in a comparison of malignant hyperthermia and normal porcine RYR1 cDNAs; the linkage of this mutation to malignant hyperthermia in over 450 pigs in six breeds, including 338 meioses; and the appearance of the corresponding mutation, Cys for Arg614, across a species barrier, in a few human families, where it also cosegregates with malignant hyperthermia. Linkage of malignant hyperthermia to RYR1 is, however, not observed in all human families with malignant hyperthermia. Accordingly, other abnormal genes that may cause the condition are being sought.
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PMID:The genetic basis of malignant hyperthermia. 132 95

Propofol is an intravenous (IV) drug recently introduced into the United States for induction and maintenance of anesthesia. In spite of extensive laboratory evaluation, it is not possible to predict all the potential side effects that might be associated with a new drug. Because malignant hyperthermia (MH) remains a serious and potentially life-threatening complication of anesthesia, all new anesthetic drugs should be considered potential triggering drugs until proven otherwise. We report the use of IV propofol for the induction and maintenance of general anesthesia in an MH patient and review the literature on this subject.
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PMID:Propofol in patients susceptible to malignant hyperthermia: a case report and review of the literature. 135 35

We report 2 patients programmed for elective surgery who were considered susceptible to develop the malignant hyperthermia syndrome. The first patient had two familiarly antecedents of death produced by postanesthetic malignant hyperthermia, whereas the second patient suffered a clinical picture compatible with this syndrome during tonsillectomy, although followed a favourable recovery. The preoperative study including measurement of muscular enzymes, isoenzymes, and muscular biopsy was normal. In both patients an intravenous prophylactic treatment with dantrolene 2.5 mg.kg1 was undertaken before surgery. The first patient underwent hysterectomy under intradural anesthesia with bupivacaine 0.5%. The second patient had a maxillary fracture which was treated with osteosynthesis under general anesthesia. During the intervention and the postoperative follow-up, both patients were free of signs related to malignant hyperthermia. We conclude that prophylaxis with dantrolene is necessary in patients susceptible to develop the syndrome of malignant hyperthermia.
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PMID:[Prophylaxis with dantrolene sodium in 2 patients with susceptibility to malignant hyperthermia]. 141 Jul 53


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