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首页> 外文期刊>Sao Paulo Medical Journal >Progressive muscular dystrophy: Duchenne type. Controversies of the kinesitherapy treatment
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Progressive muscular dystrophy: Duchenne type. Controversies of the kinesitherapy treatment

机译：进行性肌营养不良：Duchenne型。运动疗法的争议

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The authors carried out a study of children with progressive muscular dystrophy of Duchenne type (DMD), giving special attention to physiatrical follow-up, having in mind that the practice of exercises has been debated very much in the specialized literature. The goal of this study is to try to settle the limits for the utilization of kinesitherapy which should be applied only in specific situations, such as: after skeletal muscular trauma or when the respiratory system is at risk. In this situation the physiatrical procedure would be to restrict physical activity, with early use of wheelchairs and the exclusion of the use of orthoses for orthostatism. DMD, at present, has been considered a result of duplication (60%), deletion (5 to 6%) or point mutations at gen Xp21 (Zatz, 1994), that codifies a protein called Dystrophin ( Hoffman et al., 1987). Dystrophin is a cytoskeletal sarcolemmic protein that constitutes about .002% of the total protein of the muscle, present in skeletal fibers concentrated in muscle tendinous joints, which supplies mechanical reinforcement to the surface of the membrane during stretching and shortening physical activity. This protein is absent in DMD cases, wherefore, the sarcolemma undergoes a segmentary necrosis losing its contractile property during eccentric and concentric physical activity. The importance of physiatrical follow-up for DMD patients is to avoid deformities and tendon shortening, to ameliorate the patient's quality of life, to provide respiratory assistance and general couseling to members of the patient's family. The objective of this study is to try to clarify the risks and possibilities of kinesitherapy applied to DMD cases.
机译：作者进行了一项针对Duchenne型进行性肌营养不良症（DMD）的儿童的研究，特别注意生理随访，同时考虑到锻炼的方法在专业文献中已引起很大争议。这项研究的目的是尝试确定仅在特定情况下才可使用运动疗法的限制，例如：骨骼肌创伤后或呼吸系统处于危险之中。在这种情况下，应尽早采取物理行动，以限制身体活动，并应尽早使用轮椅，并排除使用矫形器进行矫正。目前，DMD被认为是Xp21世代重复（60％），缺失（5％至6％）或点突变的结果（Zatz，1994），该蛋白编码称为Dystrophin的蛋白质（Hoffman et al。，1987）。 。肌营养不良蛋白是一种细胞骨架肌膜粘膜蛋白，约占肌肉总蛋白的0.002％，存在于集中在肌腱关节的骨骼纤维中，在拉伸和缩短身体活动时为膜表面提供机械增强作用。该蛋白在DMD病例中不存在，因此，肌膜发生节段性坏死，在偏心和同心体育活动中丧失了其收缩特性。对于DMD患者，进行物理随访的重要性在于避免畸形和肌腱缩短，改善患者的生活质量，为患者家庭成员提供呼吸帮助和一般帮助。这项研究的目的是试图阐明在DMD病例中进行运动疗法的风险和可能性。

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