TY - T1的肺功能在初级纤毛dyskinesia: breaking the myth that this is a mild disease JF - European Respiratory Journal JO - Eur Respir J DO - 10.1183/13993003.01365-2018 VL - 52 IS - 2 SP - 1801365 AU - Saglani, Sejal Y1 - 2018/08/01 UR - //www.qdcxjkg.com/content/52/2/1801365.abstract N2 - Primary ciliary dyskinesia (PCD) is a chronic suppurative lung disease that is recessively inherited and has marked clinical phenotypic heterogeneity [1]. The autosomal recessive inheritance and impaired mucociliary clearance secondary to ciliary dysfunction mean the condition is often compared to cystic fibrosis both in terms of outcomes [2] and management [3]. Indeed, even though both are chronic suppurative lung diseases associated with bronchiectasis, to date PCD has often been considered mild and relatively benign compared to cystic fibrosis.Early diagnosis and intervention is essential to prevent progressive lung damage in primary ciliary dyskinesia http://ow.ly/JMCE30l8YVa ER -