Division of Pulmonology, The Children's Hospital of Philadelphia, The Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania, USA.
Pediatr Pulmonol. 2021 Apr;56(4):693-699. doi: 10.1002/ppul.25182. Epub 2020 Dec 8.
Progressive neuromuscular disease leads to muscle weakness or failure that produces loss of pulmonary function and clinical respiratory morbidity. Tracking pulmonary function in a practical and effective way is very important because it can help identify a stage of disease when a morbidity, such as inadequate airway clearance or respiratory failure, may be present. There are four general categories of pulmonary function outcome measures such as volume, flow, pressure, and gas exchange. These outcome measures have variable precision and accuracy in predicting clinical change, and practicality in performing them relative to age and condition. It is widely recommended to follow multiple measurements longitudinally and create an accurate and timely clinical picture. This manuscript will review the most commonly used and most practical measures for use in clinical practice and how they can help to assess morbidity, disease state, and help optimize patient management.
进行性神经肌肉疾病可导致肌肉无力或衰竭，从而导致肺功能丧失和临床呼吸系统发病率。以实用有效的方式跟踪肺功能非常重要，因为它可以帮助确定疾病的阶段，此时可能存在发病率，例如气道清除不足或呼吸衰竭。肺功能的结果测量有四个一般类别，如体积、流量、压力和气体交换。这些结果测量在预测临床变化方面的精度和准确性不同，并且相对于年龄和条件而言，它们在执行方面的实用性也不同。广泛建议进行多次纵向测量并创建准确及时的临床图像。本文将回顾在临床实践中最常用和最实用的测量方法，以及它们如何帮助评估发病率、疾病状态并帮助优化患者管理。
