Perelman School of Medicine at the University of Pennsylvania, Division of Pulmonary Medicine, The Children's Hospital of Philadelphia, 3501 Civic Center Boulevard, Philadelphia, PA 19104, United States.
Paediatr Respir Rev. 2019 Apr;30:9-12. doi: 10.1016/j.prrv.2018.08.004. Epub 2018 Oct 4.
In Duchenne muscular dystrophy there is a well-established progressive loss of upper and lower extremity muscle function that is evident from the earliest stages of decline and through permanent loss of function. There is not the same visible evidence of decline in respiratory muscle function decline toward respiratory failure; therefore, comprehensive pulmonary function testing provides a critical component of a comprehensive longitudinal respiratory assessment. There are multiple ways of assessing separate inspiratory and expiratory muscle function and also a summative output of each to provide broad information to help identify disease status and guide intervention as appropriate.
在杜氏肌营养不良症中，上肢和下肢肌肉功能会逐渐明显丧失，这从疾病的早期阶段就开始显现，而且会一直持续到功能永久丧失。呼吸肌功能的下降并没有同样明显的迹象表明会发展为呼吸衰竭；因此，全面的肺功能测试是全面纵向呼吸评估的重要组成部分。有多种方法可以评估单独的吸气和呼气肌肉功能，也可以综合评估它们的输出，提供广泛的信息，以帮助确定疾病状态并进行适当的干预。
