Leeds Teaching Hospitals, Leeds General Infirmary, Great George Street, Leeds LS1 3EX, UK.
Leeds Institute of Health Sciences, University of Leeds, UK.
Paediatr Respir Rev. 2018 Jan;25:73-77. doi: 10.1016/j.prrv.2017.03.011. Epub 2017 Mar 16.
Primary Ciliary Dyskinesia (PCD) is a rare inherited disease with impaired mucociliary clearance. Airway clearance techniques (ACTs) are commonly recommended for patients with PCD to facilitate mucus clearance, despite a lack of evidence in this group. Current physiotherapy practice in PCD is based on evidence extrapolated from the field of Cystic Fibrosis (CF). This paper focuses on the available evidence and outlines challenges in extrapolating evidence between the conditions for best clinical practice.
原发性纤毛运动障碍（PCD）是一种罕见的遗传性疾病，其特征为黏液纤毛清除功能障碍。气道廓清技术（ACT）常用于 PCD 患者，以促进黏液清除，但这一人群缺乏相关证据。目前 PCD 的物理治疗实践是基于从囊性纤维化（CF）领域推断出的证据。本文重点关注现有证据，并概述了在两种疾病之间推断证据以获得最佳临床实践的挑战。
