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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 now recognized as a distinct entity in anesthetic practice and can be considered as a pharmacogenetic disease of obscure etiology occuring in man and pigs with a dominant inheritance. A close association with myopathy has been noted. Commonly used muscle relaxants or anesthetic drugs can act as triggering agents in genetically susceptible patients, who develop a real hypermetabolic state, characterized by a rapid rise in body temperature, muscular rigidity, tachycardia and tachypnoea, cyanosis and severe respiratory and metabolic acidosis, the lethality being about 60%. Other clinical, biochemical and histopathological features of this condition are described. The prevention and early diagnosis of this syndrome is very important. Therefore, it is necessary in the preanesthetic evaluation, to obtain information from the patient, with regard to previous anesthetic experiences, and to have a more exact anamnesis in patients with muscular diseases or with other members of the family under suspicion. Some screening methods are described. The prognosis of malignant hyperthermia depends on an early diagnosis. Although the incidence is, fortunately, small, this condition is sufficiently significant and acute in nature to require that anesthesiologist be aware of its clinical pathophysiology and prepared to recognize and treat it promptly. Therefore body temperature should be controlled continuously in all anesthetized patients, particularly in the younger age group and especially in those in which symptoms of muscle rigor have been observed particularly after application of succinylcholine and halothane. A regime of treatment is suggested, based on current concepts of the pathogenesis. It consists in establishing effective and rapid cooling, reversal of tissue hypoxia and correction of respiratory and metabolic acidosis and hyperkalemia. Specific therapy with dantrolene sodium may prove to be an answer to this serious problem.
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PMID:Malignant hyperthermia. 70 24

Malignant hyperthermia is a rare but severe complication of modern anesthesia, induced by halothane and succinylcholine. The syndrome is characterized by a rapid sustained and extreme rise in body temperature associated with muscular rigidity, tachycardia, tachypnoea and cyanosis. The lethality is about 60%. The present paper describes the histological, histochemical and electron microscopical findings performed on muscle biopsies of 3 patients with malignant hyperthermia (1 patient died) and a so called risk patient. In all patients morphological findings consistent with a pre-existent myopathy were found. Histologically there were acute necrotic muscular fibers as well as in types I and II, variations in the fiber diameter and centralization of the nuclei. In two cases even fibers that had a normal aspect in HE slides, showed a pathologic pattern after phosphorylase reaction. In addition to acute rhabdomyolysis, electron-microscopic investigations revealed cystic expansion of the cisterns of the sarcoplasmic reticulum with a peculiar proliferation of the sarcolemma. In a degenerating mitochondrium, a crystalline inclusion was identified. These findings support the pathogenetic concept of Britt and coworkers of a functional defect in the calcium release or binding mechanism of sarcoplasmic reticulum. Since it is known that malignant hyperthermia has a familial predilection, it seems very important that clinical, biochemical, and morphological investigations be performed such as CPK estimations and muscular biopsies not only of the patients but also of the relatives in order to rule out this type of latent myopathy.
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PMID:[Histological, histochemical, and ultrastructural findings in malignant hyperthermia (author's transl)]. 80 99

Two cases of malignant hyperthermia with different clinical courses are reported. The patients showed the classical signs of malignant hyperthermia consisting of tachycardia, tachypnoea, ocasional peripheral cyanosis, high body temperature as well as characteristic rise in serum enzymes. In one of the patients the symptoms were recognized early during the operation. The immediate commencement of therapy with ice-cooled. Ringer-Lactate-Solution, Procainmedication, Corticoids as well as physical body cooling favourably influenced the clinical course and the patient survived. In both cases the patients underwent succinylcholine and halothane anaesthesia, but the symptoms of the second patient appeared after the reduction of anaesthesia. In spite of vigorous therapy the hyperpyrexia resulted in heart arrest and death. Morphologically, both patients showed signs of preexistent myopathy with volumetric alterations of the muscle fibres, centralisation of the nuclei and acute muscle fibre necrosis. On the basis of the observed variable course, the various symptom complexes reported in the literature to data are reviewed. A detailed discussion of the "carrier problem" and the available treatment possibilities is also made. Realising that malignant hyperthermia is an inheritable disease, prophylactic measures such as, f.i. the issue of medical certificates to the patient and his relatives are suggested.
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PMID:[Report on 2 cases of malignant hyperthermia with different clinical courses]. 96 90

Malignant hyperthermia is a life-threatening complication of general anesthesia. Its cause is not precisely known but it appears to be related to a genetic defect that allows increased release or decreased reaccumulation of calcium by the sarcoplasmic reticulum whech then results in a hypermetabolic state. As with any unexpected complication when a patient is under general anesthesia, early diagnosis and treatment are essential. The early clinical signs that the surgeon and anesthesiologist shoulc be alert to are unexplained tachycardia, unexplained tachypnea, muscular rigidity, and increased temperature. Therapy should be accurate and immediate. The essentials of therapy are discontinuance of the anesthetic agent; immediate, active, and aggressive cooling; administration of procaine or procaine amide, 1 mg/kg/min until the pulse slows; correction of electrolyte and acid-base imbalances; maintenance of urinary output with furosemide and large volumes of fluids, intravenously; and supportive care. A thorough knowledge of the management of malignant hyperthermia ahd the pathophysiology of the complications that may occur with general anesthesia will allow the oral surgeon to fully meet his obligations to his patients.
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PMID:Survival of an oral surgery patient with malignant hyperthermia. 105 44

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

Heat stroke victims lack thermoregulatory control. Treatment includes immediate cooling, circulatory support and monitoring for secondary complications. Neuroleptic malignant syndrome is a complication of neuroleptic drug therapy; skeletal muscle hypertonicity helps distinguish this entity from heat stroke. Malignant hyperthermia should be considered in any patient who is under physiologic or anesthetic stress and develops hyperthermia plus skeletal muscle rigidity, tachypnea, hypoxia, tachycardia and hyperkalemia.
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PMID:Hyperthermic syndromes. 328 39

Malignant hyperthermia (MH) may be triggered by exposure to commonly employed anesthetic agents and muscle relaxants, and often manifests itself during the period of anesthesia. Delayed-onset MH occurring one to four hours postoperatively has been described in isolated case reports. A case of delayed-onset MH occurred 11 hours following routine tonsillectomy and adenoidectomy. The patient demonstrated tachypnea, tachycardia, hyperthermia, and metabolic acidosis. Prompt intravenous administration of dantrolene sodium was therapeutic. Serial serum creatine phosphokinase evaluation verified the diagnosis of MH. The implications of delayed-onset MH and the importance of preoperative screening for potentially susceptible individuals are discussed.
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PMID:Markedly delayed postoperative malignant hyperthermia. 395 99

Malignant hyperthermia has been an iatrogenic syndrome which was usually fatal. The syndrome occurs when certain physiologically active compounds act on defective skeletal and cardiac muscle cells. The syndrome consists of a soaring fever, severe acidosis, tachycardia, tachypnea, and usually myoclonic spasms. Cardiac arrhythmias, shock, bleeding disorders, and death soon follow. Malignant hyperthermia has characteristically complicated the administrations of anesthesiologists, but is being triggered by drugs used in other diagnostic and therapeutic activities. This paper reports a fatal case which followed the infusion of iodinated contrast media. The increased release of epinephrine and the production of fibrin split products seen in an iodinated contrast media reaction suggest certain commonalities between it and a malignant hyperthermia reaction which may be triggered be epinephrine and is complicated by disseminated vascular clotting and bleeding disorders. The potential for successful treatment has greatly improved with the availability of dantrolene. Increased awareness of the syndrome, temperature monitoring, early diagnosis, and rapid treatment should make this malignant disorder less threatening.
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PMID:Malignant hyperthermia following intravenous iodinated contrast media. Report of a fatal case. 701 55

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

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


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