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

This communication presents evidence in support of a neuropathic basis for the myopathy associated with malignant hyperpyrexia (MH). Muscle from MH susceptible individuals showed a reduced calcium uptake by the sarcoplasmic reticulum. There was a reduced concentration of phosphocreatine and ATP and an increased concentration of glucose-6-phosphate in these muscle samples.
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PMID:Morphological and biochemical defects in muscles of human carriers of the malignant hyperthermia syndrome. 12 80

Virus-like particles (VLP) have been identified by electronmicroscopy in the skeletal muscles (paraxials) of six cases of idiopathic scoliosis. These particles closely resembled VLP reported in the skeletal muscles in other conditions, e.g. Reye's syndrome, polymyositis, malignant hyperthermia, and chronic myopathy. We have shown by specific staining that these structures are composed of glycogen in a crystalline form. Using Coxsackie B infected tissue culture cells as a control we have shown that these viruses, which are of similar shape and size to the VLP, were unstained using this specific staining method.
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PMID:The nature of virus-like particles in the paraxial muscles of idiopathic scoliosis. 23 Mar 31

Malignant hyperthermia is a dominantly inherited, usually subclinical, disease that occurs in individuals who have an underlying muscular disorder and connotes the gravest possible consequences. When it occurs, it is usually during the use of muscle relaxants in anesthesia and potent anesthetic agents such as halothane. Patients at risk must be identified through careful history and screening procedures; however, a patient susceptible to this condition may have had general anesthesia in the past without complications. A careful monitoring regimen must be established for the procedure and some means of cooling the patient must be ready in case pyrexia occurs. Dantrolene sodium is currently the preferred drug for prevention of the syndrome and may be valuable for its treatment.
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PMID:Dantrolene sodium in the management of patients at risk from malignant hyperthermia. 28 34

Malignant hyperthermia is an inherited muscular disorder and a cause of sudden death in afflicted patients. Stress arising from tramuma, surgery, and other causes can initiate the syndrome. We studied the types of orthopaedic problems commonly seen in patients with this condition as well as the clinical manifestations and pathophysiology of the syndrome. Recognition, management, and understanding of the pathophysiology of malignant hyperthermia can be aided by a study of its similarities to porcine stress syndrome. We performed experimental studies of muscle and prophylaxis in swine. We also demonstrated the extension of the prophylactic use of dantrolene sodium in a management protocol in eighty surgical procedures in humans.
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PMID:Malignant hyperthermia. A potentially fatal syndrome in orthopaedic patients. 48 49

An eight-year-old male suffered from long-standing proximal muscle weakness, dramatically aggravated by febrile episodes. Neuromuscular work-up disclosed a myopathy with multiple central cores of non-familial nature. The presence of central cores in this patient as well as in the myopathy of malignant hyperpyrexia might suggest a pathophysiological basis common to both conditions.
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PMID:Myopathy with multiple central cores. A case with hypersensitivity to pyrexia. 58 99

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

General anaesthesia with Althesin was administered on two occasions to a patient who was identified as susceptible to malignant hyperthermia, in whom there was identified familial subclinical myopathy and once in another patient suffering from arthrogryposis multiplex congenita with a history of fever associated with two previous anaesthetics. In the first patient halothane was administered by accident in association with the Althesin, but no hyperpyrexia occurred. In the second instance nitrous oxide-oxygen and halothane were associated purposely with Althesin. In none of these cases was there any rise in temperature, muscle rigor or elevation of the serum CPK level. This experience corroborates the experimental evidence of Hall, et al.10 and Harrison, who reported that Althesin prevented the onset of hyperthermia, and the clinical reports of Page and Judelman. Althesin can be assumed to be an effective anaesthetic for malignant hyperthermia susceptible patients.
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PMID:Malignant hyperthermia and althesin. 89 May 63

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

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


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