MALIGNANT HYPERTHERMIA: HISTORY OR REALITY
Keywords:
Anesthesia; Malignant Hyperthermia; In vitro contracture test; dantroleneAbstract
Introduction. Malignant Hyperthermia (MH) is known as an anaesthetic risk since 1960. Morbidity has decreased remarkably with better understanding of the pathophysiology and availability of a specific treatment, but still patients are dying of a fulminant MH crisis. Case Presentation. We report a male patient undergoing general anaesthesia with propofol, isoflurane and atracurium for appendectomy developing Malignant Hyperthermia. The boy dies of multiple organ failure in unavailability of dantrolene. As the parents could not afford the far travel to a MH laboratory to undergo an IVCT (In vitro contracture test) we did a genetic analysis of their blood. The results of mutation screening showed no mutations in the father and a mutation in exon 40: 6635 T>C Val-2212-Alnine. This mutation is not accepted as causative by the European Malignant Hyperthermia Organisation . Conclusions. Crisis of MH is a rare event with a serious risk of lethal outcome for susceptible individuals. In order to keep mortality low, meticulous preoperative evaluation of patient medical history, adequate intraoperative monitoring, availability of a sufficient stock of dantrolene and thorough training of anesthetists and theatre staff need to be provided by any institution delivering general anaesthesia.
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