[Malignant hyperthermia].
Malignant hyperthermia is an autosomal dominant disorder of the skeletal muscle that predisposes affected individuals to a life-threatening hypermetabolic reaction in response to volatile anaesthetics and depolarizing muscle relaxants. The underlying heterogeneous genetic defects are mainly point mutations within the ryanodine receptor gene of the sarcoplasmic reticulum. Following the introduction of efficient diagnostic and therapeutic tools--the in vitro contracture test and intravenous treatment with dantrolene--a dramatic decline in mortality rates has been observed. The association of malignant hyperthermia-like reactions with other neuromuscular disorders requires the collaboration of several clinical disciplines to achieve a timely recognition of this still life-threatening disorder.
Zimprich
Fritz
F
Universitätsklinik für Neurologie, Wien, Osterreich.
Kress
Hans Georg
HG
Zeitlhofer
Josef
J
D000328:Adult; D001706:Biopsy; D002648:Child; D003620:Dantrolene / Q000008:administration & dosage / Q000627:therapeutic use; D006801:Humans; D007275:Injections, Intravenous; D008305:Malignant Hyperthermia / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology / Q000235:genetics / Q000401:mortality / Q000473:pathology; D009119:Muscle Contraction; D009125:Muscle Relaxants, Central / Q000008:administration & dosage / Q000627:therapeutic use; D018482:Muscle, Skeletal / Q000187:drug effects / Q000473:pathology; D017354:Point Mutation; D012307:Risk Factors; D019837:Ryanodine Receptor Calcium Release Channel / Q000235:genetics; D012519:Sarcoplasmic Reticulum / Q000235:genetics; D012680:Sensitivity and Specificity
D009125:Muscle Relaxants, Central; D019837:Ryanodine Receptor Calcium Release Channel; D003620:Dantrolene