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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 (MH) is a chemically induced, genetic myopathy characterized by metabolic and respiratory acidosis, arrhythmias, hyperpyrexia, and muscular rigidity usually occurring during or within 24 hours postanesthesia. It is initiated by some anesthetic drugs, muscle relaxants, and possibly by catecholamines produced by stress. The incidence of the disease is 1:15,000 in populations who have received a general anesthetic. A recently developed platelet bioassay test allows for rapid identification of the susceptible individual, while medication with dantrolene sodium can usually prevent or reverse the syndrome.
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PMID:Current concepts of malignant hyperthermia. 680 85

It has been suspected that the cause of malignant hyperthermia (MH) is an abnormality in the sarcoplasmic reticulum of skeletal muscle. We isolated the sarcoplasmic reticulum from malignant hyperthermia-susceptible (MHS) patients and controls and analysed the protein composition with sodium dodecyl sulfate polyacrylamide gel electrophoresis. There were no remarkable changes in the sarcoplasmic reticulum protein composition profile of the scanned gel of the patients. Quantitative measurement of the relative proportion of each band in the gel, however, revealed a slight decrease in calsequestrin and a slight increase in protein of molecular weight 23,000. (Ca2+ -Mg2+)ATPase had no altered subfragments in MHS patients. Crude mitochondrial proteins and myoplasmic proteins showed minor alterations in composition in some patients. The data supported the thesis that malignant hyperthermia is due to defects in several different cell membranes including the sarcoplasmic reticulum and the mitochondria.
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PMID:Analysis of sarcoplasmic reticulum proteins in patients susceptible to malignant hyperthermia. 687 11

Anesthesiologists from 65 institutions participated in a multicenter study to assess the efficacy of lyophilized intravenous dantrolene sodium in treating anesthetically related malignant hyperthermia (MH). Of 21 patients treated with the drug, eight were judged to have unequivocal MH and were treated according to study protocol. Three were judged to have probable MH and were also treated according to study protocol. All 11 recovered without sequelae from MH and without adverse drug effects. A mean dantrolene dose of 2.5 mg/kg in these patients produced significant changes in clinical and biochemical parameters suggestive of decreased cellular metabolism. Four patients with unequivocal MH were treated with intravenous dantrolene more than 24 h after the diagnosis of MH; this delay in treatment of clinical signs in these patients, the mortality rate was 75 per cent, which is comparable to that reported without dantrolene. The six remaining patients had episodes of questionable MH during or subsequent to anesthesia and were treated with dantrolene. There was insufficient evidence to justify an unequivocal or probable diagnosis of MH, and they, therefore, were not included in the study. All survived and had no adverse drug reactions. Dantrolene therapy resulted in a statistically significantly lower mortality rate than would be expected in MH patients. The study supports animal data suggesting that dantrolene is specific in reversing MH.
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PMID:Dantrolene in human malignant hyperthermia. 703 19

Five subjects, all relatives of two patients who suffered from malignant hyperthermia, were tested for blood CK activity before and after a course of dantrolene sodium. (4 mg/kg/day orally for three days.) In all the cases, the CK levels which were significantly elevated prior to the drug administration, dropped to normal values after three days of treatment returning to the original levels after eight days. The effect of the drug is most likely related to a temporary normalisation of muscle metabolism which may prevent the development of malignant hyperthermia in susceptible patients. The blood CK activity can be a valuable indicator in determining the effective dose of dantrolene. Prophylactic drug therapy should be considered even when special anesthesia techniques are used.
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PMID:[Transient normalisation of CK blood levels in patients susceptible to malignant hyperthermia by oral administration of dantrolene (author's transl)]. 710 34

During induction with volatile anaesthetic agents and succinylcholine (suxamethonium) both children showed a singular clinical symptom: trismus. In the first case(1) narcosis had been interrupted followed by clinically controlled recovery. One week later, after treatment with oral dantrolen (Dantamacrin), anaesthesia with triggerfree agents was performed without complications. In the second case (2) anaesthesia has been continued by using triggerfree agents. Both children showed significant increases of Creatin-Kinase-activities over 5000 U/l after the interrupted narcosis (1) and postoperatively as being typical biochemical parameters for incipient hyperthermia maligna. All other parameters were normal. A review is given on the use of dantrolene-sodium, its development and its use in our department as lifesaving agent in malignant hyperthermia.
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PMID:[Malignant hyperthermia. Current aspects of dantrolene treatment of 2 cases of hyperthermia maligna incipiens]. 712 60

A case of malignant hyperthermia in a young girl with vesicoureteral reflux and multiple congenital abnormalities is discussed. Malignant hyperthermia is a relatively newly defined problem that is associated with anesthesia. It is being recognized with increasing frequency, particularly in children with multiple musculoskeletal anomalies. The term itself is a misnomer. It is a drug-induced syndrome of hypermetabolism that may represent an inborn error of metabolism. Recognition, prophylaxis and crisis management are reviewed, and the use of dantrolene sodium in the care of patients with malignant hyperthermia is discussed. Because recognition of malignant hyperthermia is an important concern for the practicing urologist, the principles of identifying the patient at risk, as well as managing the crisis, should be familiar to the clinician.
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PMID:Management of malignant hyperthermia. 721 71

Malignant hyperpyrexia (MH) is a syndrome with a mortality of 60%, triggered by a broad variety of anesthetic agents. The most significant recent advance influencing the clinician's ability to safely manage MH individuals is the emergence of dantrolene sodium as both a prophylactic and a therapeutic agent. The purpose of this presentation is to review recent clinical and laboratory studies and advances which are the basis for establishing the pathophysiology of this syndrome and pharmacology of treatment. The FDA has recently approve intravenous Dantrium for treatment of malignant hyperpyrexia. Intravenous and oral Dantrium has much theoretical support and experimental evidence which suggests it will significantly reduce the mortality previously associated with this syndrome.
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PMID:Malignant hyperpyrexia: an update for the otolaryngologist. 724 87

Malignant hyperthermia, a relatively recently described entity, is a little-understood disease process usually manifesting as operative or postoperative hyperpyrexia in association with a hypermetabolic state. Specific therapy with procaine (Novocaine) and more recently with a muscle relaxant, dantrolene sodium (Dantrium), has shown itself to be life-saving, and currently diagnosis can be made by muscle biopsy in patients from affected pedigrees. Malignant hyperthermia is a risk in all general anesthetic procedures, particularly squint and ptosis repair, and may even be a consideration with local anesthesia.
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PMID:Malignant hyperthermia. Considerations for the ophthalmologist. 724 23

Malignant hyperthermia is an autosomal dominant disorder with variable expressivity that is caused by a membrane defect in the sarcolemma of myofibrils. A patient with strabismus (esotropia) had tachycardia and masseter muscle rigidity on exposure to succinylcholine chloride and halothane, but because of rapid recognition of the condition and discontinuation of the procedure, the potentially lethal complications of malignant hyperthermia did not develop. A serum creatine phosphokinase level showed a substantial increase above normal. Two weeks later, the patient underwent successful correction of the strabismus under general anesthesia, using morphine sulfate and thiopental sodium without complication. This condition is of interest to ophthalmologists because it occurs with increasing frequency in patients with strabismus and ptosis, and it may be triggered by certain local anesthetic agents often used by ophthalmologists.
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PMID:Suspected malignant hyperthermia in a strabismus patient. A case report. 725 98

The incidence, etiology, clinical manifestations, and management of malignant hyperthermia (MH) are reviewed. The syndrome of MH is recognized as one of the causes of anesthesia-related deaths. It is considered pharmacogenetic because both an abnormal gene and precipitating environmental factors are necessary to produce an acute reaction. Metabolic defects, involving a derangement of calcium dynamics, appear to be the common characteristic of susceptible individuals. Calcium release and uptake from the sarcoplasmic reticulum is altered when an individual with MH is exposed to certain anesthetic agents or triggering physical and emotional stresses. Muscle rigidity, tachycardia, tachypnea, and high fever can lead to other complications and death. Management of an acute reaction of MH includes cooling methods to lower body temperature, hyperventilation, sodium bicarbonate control of acidosis, maintenance of fluid and electrolyte balance, and the administration of dantrolene sodium. A thorough family history, baseline CPKs, caffeine-halothane contracture tests, and ultramicroscopic examination of muscle biopsy specimens are recommended as screening techniques. The early administration of dantrolene sodium in acute reaction of MH has been shown to rapidly alleviate the symptoms and ensuing severe complications. Individuals with a strong family history of MH or previous episodes may be treated with oral dantrolene sodium before surgery to effectively prevent a crisis, and after surgery to prevent recurrence.
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PMID:Malignant hyperthermia: current perspectives. 728 92


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