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- W2970898089 abstract "Muscular dystrophy (MD) refers to a group of hereditary diseases characterized by painless degeneration and loss-of-function of skeletal muscles. Out of these, the X-linked recessive types such as Duchenne (DMD) and Becker muscular dystrophies (BMD) remain the most common subtypes. Both DMD and BMD result from a deficiency of the muscle-stabilizing protein dystrophin, whose expression is either completely lost (as in DMD) or dysfunctional (as in BMD). This, in turn, results in weakness and motor delay that eventually proves to be fatal from respiratory failure. Anesthetic management of patients with DMD appears complex with several critical considerations. First, loss of functioning dystrophin leads to instability of the sarcolemma, encouraging constant Ca2+ leakage into the cytoplasm that leads not only to activation of proteolytic enzymes but also sensitizes the cells to further Ca2+ increases. Administration of inhalation agents like sevoflurane or paralyzing agents like succinylcholine raise intracellular Ca2+ levels and have resulted in rhabdomyolysis, hypermetabolic hyperthermia, as well as hyperkalemia with risks of myocardial infarction. Second, patient positioning also proves to be problematic as atrophy paralyzes the limbs in awkward, inaccessible postures that limit surgical visibility." @default.
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- W2970898089 date "2019-09-01" @default.
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- W2970898089 title "Anesthetic Management of Patients with Hereditary Muscular Dystrophin Disorders" @default.
- W2970898089 doi "https://doi.org/10.1016/j.joms.2019.06.082" @default.
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