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Perioperative Management of a Patient With Giant Axonal Neuropathy

机译:巨大轴索神经病患者的围手术期管理

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Giant axonal neuropathy (GAN) is a rare, autosomal recessive, progressive neurodegenerative disorder affecting both the central and peripheral nervous systems. The aberrant accumulation of cytoskeletal-associated proteins causes neurodegeneration resulting in both motor and sensory dysfunction. Given the invariable and progressive involvement of the neuromuscular system, anesthetic care is frequently needed to correct the orthopedic sequelae of the disorder. We present a 12-year-old girl with GAN who required anesthetic care for posterior spinal fusion to treat scoliosis. Previous reports of anesthetic care for patients with GAN are reviewed, and the perioperative considerations of these patients are discussed.J Med Cases. 2015;6(11):512-516doi: http://dx.doi.org/10.14740/jmc2346w
机译:巨大轴突神经病(GAN)是一种罕见的常染色体隐性遗传,进行性神经退行性疾病,会影响中枢神经系统和周围神经系统。细胞骨架相关蛋白的异常积累导致神经变性,导致运动和感觉功能障碍。考虑到神经肌肉系统的不断累进,经常需要麻醉护理来矫正该疾病的骨科后遗症。我们介绍了一名12岁的GAN女孩,需要麻醉后进行脊柱融合治疗脊柱侧弯。回顾了以前对GAN患者进行麻醉护理的报道,并讨论了这些患者的围手术期考虑因素。 2015; 6(11):512-516doi:http://dx.doi.org/10.14740/jmc2346w

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