Orthopedic management of the muscular dystrophies.
Muscular dystrophy is a collective group of inherited, noninflammatory, progressive muscle wasting diseases. The initial pathologic feature is an abnormality in the genetic code for dystrophin or one of its associated glycoproteins, which leads to the various clinical syndromes. Despite minor variations between the different types, all muscular dystrophies have in common progressive muscle weakness, which is best typified by Duchenne muscular dystrophy. The weakness occurs in a proximal to distal direction and can compromise ambulatory status as well as cardiopulmonary function. Additionally, structural soft tissue contractures and spinal deformities may develop from poor posturing secondary to the progressive muscle weakness and imbalance. The rapidly developing scoliosis and its associated pelvic obliquity can even compromise sitting. Recent advances in molecular biology and gene therapy research raise the hope for a cure for muscular dystrophy in the near future. Until that time, however, the role of orthopedic surgeons in treating patients with muscular dystrophy is to preserve or prolong their functional status for as long as possible. This can be achieved by physical therapy, bracing, soft tissue releases for joint contractures, and early stabilization of the spine.
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Twee
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Children's Hospital Medical Center, Cincinnati, Ohio 45229, USA. tweedo@chmcc.org
D002648:Child; D003286:Contracture / Q000209:etiology / Q000601:surgery; D006801:Humans; D009136:Muscular Dystrophies / Q000601:surgery*; D019637:Orthopedic Procedures; D012600:Scoliosis / Q000209:etiology / Q000601:surgery