Malignant hyperpyrexia during anaesthesia: possible association with subclinical myopathy. 1970

H Isaacs, and M B Barlow

The members of a family in whom three malignant hyperpyrexial deaths occurred during anaesthesia were studied by means of serum creatine phosphokinase estimations. Abnormally high levels were found in many asymptomatic relatives. It is suggested that the abnormal levels reflect a subclinical myopathy of autosomal dominant inheritance which possesses a potentially lethal propensity resulting in a malignant hyperpyrexia when challenged with various anaesthetic agents. Possibly sudden unexplained deaths under varying circumstances are a further expression of this underlying abnormality.A possible clue to the anticipation of future cases of malignant hyperpyrexia has been found, and it is suggested that relatives of previous cases be investigated.

UI MeSH Term Description Entries
D008297 Male Males
D009135 Muscular Diseases Acquired, familial, and congenital disorders of SKELETAL MUSCLE and SMOOTH MUSCLE. Muscle Disorders,Myopathies,Myopathic Conditions,Muscle Disorder,Muscular Disease,Myopathic Condition,Myopathy
D003402 Creatine Kinase A transferase that catalyzes formation of PHOSPHOCREATINE from ATP + CREATINE. The reaction stores ATP energy as phosphocreatine. Three cytoplasmic ISOENZYMES have been identified in human tissues: the MM type from SKELETAL MUSCLE, the MB type from myocardial tissue and the BB type from nervous tissue as well as a mitochondrial isoenzyme. Macro-creatine kinase refers to creatine kinase complexed with other serum proteins. Creatine Phosphokinase,ADP Phosphocreatine Phosphotransferase,ATP Creatine Phosphotransferase,Macro-Creatine Kinase,Creatine Phosphotransferase, ATP,Kinase, Creatine,Macro Creatine Kinase,Phosphocreatine Phosphotransferase, ADP,Phosphokinase, Creatine,Phosphotransferase, ADP Phosphocreatine,Phosphotransferase, ATP Creatine
D003645 Death, Sudden The abrupt cessation of all vital bodily functions, manifested by the permanent loss of total cerebral, respiratory, and cardiovascular functions. Sudden Death
D005260 Female Females
D005334 Fever An abnormal elevation of body temperature, usually as a result of a pathologic process. Pyrexia,Fevers,Pyrexias
D005799 Genes, Dominant Genes that influence the PHENOTYPE both in the homozygous and the heterozygous state. Conditions, Dominant Genetic,Dominant Genetic Conditions,Genetic Conditions, Dominant,Condition, Dominant Genetic,Dominant Gene,Dominant Genes,Dominant Genetic Condition,Gene, Dominant,Genetic Condition, Dominant
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000769 Anesthesia, Inhalation Anesthesia caused by the breathing of anesthetic gases or vapors or by insufflating anesthetic gases or vapors into the respiratory tract. Insufflation Anesthesia,Anesthesia, Insufflation,Inhalation Anesthesia

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