Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0024591 (malignant hyperthermia)
 2,353 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Episodes of acute myoglobinuria or cardiac arrest were occasionally complicated in general anesthesia of patients with Duchenne or Becker muscular dystrophy (DMD/BMD). Whether these complications are malignant hyperthermia (MH) or not has several times been discussed. In the present study, we applied the clinical grading scale (CGS) of Larach and modified criteria of caffeine contracture test of the skinned fiber (sIVCT) to solve this problem. When the CGS was applied to reported MH-like episodes of DMD/BMD cases, 9 out of 20 cases were classed as almost certain or very likely MH. According to results of sIVCT in 11 patients with DMD/BMD, 5 patients were judged as MHS (MH-susceptible) and 3 as MHE (MH-equivocal). The diagnostic specificity of present MHS criteria was 100% for the fulminant MH. A possible "false positive" result in European IVCT has been discussed in relation to myopathy such as muscular dystrophy. When we applied our sIVCT to the muscle of mdx mouse, caffeine contracture was rather reduced compared to controls. Present study suggested that a true MH was complicated in some cases of DMD/BMD. In certain stage of muscular degeneration, patients with DMD/BMD become susceptible to MH, probably temporarily, but exact mechanism still awaits clarification.
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PMID:[Malignant hyperthermia of Duchenne muscular dystrophy: application of clinical grading scale and caffeine contracture of skinned muscle fibers]. 1100 22

Adverse reactions to genral anesthesia, which partly resembled malignant hyperthermia (MH), were more frequent in muscular dystrophy than in controls. In the present study, 35 cases so far reported in Duchenne or Becker muscular dystrophy (DMD or BMD) were analyzed and their pathogenesis was discussed. Cardiac involvements were sole manifestations in 7 cases. In other 28 cases, the acute rhabdomyolysis was the most prevailing manifestation. About 60% of myolysis cases were associated with muscle contracture (rigidity) or other hypermetabolic signs such as hypercapnia, hyperthermia and metabolic acidosis. Cases with BMD were more hyperthermic than with DMD. These results suggest Ca ion-induced hypermetabolic reactions are also present in dystrophinopathy, which have been assumed as core syndromes of the classical (gene-defined) MH. However, question whether the abnormal Ca ion is from the extracellular or intracellular stores is still unclear. Circumstancial evidences suggest that the Ca-induced Ca release (CICR) mechanism might also be involved. Endogenous redox modulators such as nitric oxide or reactive oxygen species in the dystrophic muscle might contribute to the perturbed Ca ion homeostasis.
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PMID:[Malignant hyperthermia-like reactions in Duchenne or Becker muscular dystrophy: review and hypothesis]. 1832 2