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

The muscular dystrophies are inherited myogenic disorders characterized by progressive muscle wasting and weakness of variable distribution and severity. They can be subdivided into several groups, including congenital forms, in accordance with the distribution of predominant muscle weakness: Duchenne/Becker; limb-girdle; Fukuyama type congenital muscular dystrophy; Emery-Dreifus; facioscapulohumeral; oculopharyngeal; myotonic dystrophy, et al. Muscular dystrophies are susceptible to perioperative respiratory, cardiac and other complications, such as rhabdomyolysis. Halogenated inhalational anesthetic agents have been implicated as a cause of acute rhabdomyolysis that resembles malignant hyperthermia. Depolarizing neuromuscular blocking drugs that cause rhabdomyolysis are contraindicated in these patients. Recommendations are proposed for the safe anesthetic management of these patients.
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PMID:[Anesthesia preoperative preparation of muscular dystrophy]. 2085 62

Children with neuromuscular diseases present a wide range of clinical manifestations and clinical implications for the anaesthesiologist. Neuromuscular diseases in children affect muscle strength by either directly weakening the muscle fibrils or indirectly by a degenerative nerve supply and weak neuromuscular junction. Of the more than 200 neuromuscular disorders known, the vast majority are genetic in origin. This review focuses on four of the more common neuromuscular disorders with emphasis on their pathophysiology and clinical implications for anaesthesiologists: malignant hyperthermia, the muscular dystrophies (Duchenne's, Becker's, and Emery-Dreifuss), mitochondrial disorders, and cerebral palsy.
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PMID:Perioperative management of the paediatric patient with coexisting neuromuscular disease. 2215 73