Shapiro Adam J, Zariwala Maimoona A, Ferkol Thomas, Davis Stephanie D, Sagel Scott D, Dell Sharon D, Rosenfeld Margaret, Olivier Kenneth N, Milla Carlos, Daniel Sam J, Kimple Adam J, Manion Michele, Knowles Michael R, Leigh Margaret W
Department of Pediatrics, Montreal Children's Hospital, McGill University, Quebec, Canada.
Department of Pathology and Laboratory Medicine, University of North Carolina School of Medicine, Marsico Lung Institute, Chapel Hill, North Carolina.
Pediatr Pulmonol. 2016 Feb;51(2):115-32. doi: 10.1002/ppul.23304. Epub 2015 Sep 29.
Primary ciliary dyskinesia (PCD) is a genetically heterogeneous, rare lung disease resulting in chronic oto-sino-pulmonary disease in both children and adults. Many physicians incorrectly diagnose PCD or eliminate PCD from their differential diagnosis due to inexperience with diagnostic testing methods. Thus far, all therapies used for PCD are unproven through large clinical trials. This review article outlines consensus recommendations from PCD physicians in North America who have been engaged in a PCD centered research consortium for the last 10 years. These recommendations have been adopted by the governing board of the PCD Foundation to provide guidance for PCD clinical centers for diagnostic testing, monitoring, and appropriate short and long-term therapeutics in PCD patients.
原发性纤毛运动障碍（PCD）是一种具有遗传异质性的罕见肺部疾病，可导致儿童和成人出现慢性耳-鼻-肺部疾病。许多医生由于缺乏诊断测试方法的经验，会错误地诊断PCD或在鉴别诊断中排除PCD。迄今为止，所有用于PCD的治疗方法都未经大型临床试验证实。这篇综述文章概述了北美PCD医生的共识性建议，这些医生在过去10年中一直参与一个以PCD为中心的研究联盟。PCD基金会理事会已采纳这些建议，为PCD临床中心在PCD患者的诊断测试、监测以及适当的短期和长期治疗方面提供指导。
