Lucas Jane S, Gahleitner Florian, Amorim Adelina, Boon Mieke, Brown Philippa, Constant Carolina, Cook Simon, Crowley Suzanne, Destouches Damien M S, Eber Ernst, Mussaffi Huda, Haarman Eric, Harris Amanda, Koerner-Rettberg Cordula, Kuehni Claudia E, Latzin Philipp, Loebinger Michael R, Lorent Natalie, Maitre Bernard, Moreno-Galdó Antonio, Nielsen Kim G, Özçelik Uğur, Philipsen Lue Katrine Drasbæk, Pohunek Petr, Polverino Eva, Rademacher Jessica, Robinson Phil, Snijders Deborah, Yiallouros Panayiotis, Carr Siobhán B
Primary Ciliary Dyskinesia Centre, NIHR Southampton Biomedical Research Centre, University of Southampton and University Hospital Southampton NHS Foundation Trust, Southampton, UK.
Pulmonology Dept, Centro Hospitalar S. João, Porto, Portugal.
ERJ Open Res. 2019 Feb 1;5(1). doi: 10.1183/23120541.00147-2018. eCollection 2019 Feb.
Pulmonary exacerbations are a cause of significant morbidity in patients with primary ciliary dyskinesia (PCD) and are frequently used as an outcome measure in clinical research into chronic lung diseases. So far, there has been no consensus on the definition of pulmonary exacerbations in PCD. 30 multidisciplinary experts and patients developed a consensus definition for children and adults with PCD. Following a systematic review, the panel used a modified Delphi process with a combination of face-to-face meetings and e-surveys to develop a definition that can be used in research settings for children and adults with PCD. A pulmonary exacerbation was defined by the presence of three or more of the following seven items: 1) increased cough, 2) change in sputum volume and/or colour, 3) increased shortness of breath perceived by the patient or parent, 4) decision to start or change antibiotic treatment because of perceived pulmonary symptoms, 5) malaise, tiredness, fatigue or lethargy, 6) new or increased haemoptysis, and 7) temperature >38°C. The consensus panel proposed that the definition should be used for future clinical trials. The definition should be validated and the usability assessed during these studies.
肺部加重是原发性纤毛运动障碍（PCD）患者发病的一个重要原因，并且在慢性肺部疾病的临床研究中经常被用作一项结局指标。到目前为止，关于PCD中肺部加重的定义尚未达成共识。30位多学科专家和患者为患有PCD的儿童和成人制定了一个共识定义。在进行系统综述之后，该小组采用了改良的德尔菲法，结合面对面会议和电子调查来制定一个可用于PCD儿童和成人研究的定义。肺部加重定义为出现以下七项中的三项或更多：1）咳嗽加剧，2）痰液量和/或颜色改变，3）患者或家长感觉到的呼吸急促加重，4）因感觉到肺部症状而决定开始或改变抗生素治疗，5）不适、疲倦、乏力或无精打采，6）新出现或咯血增加，7）体温>38°C。共识小组建议该定义应在未来的临床试验中使用。在这些研究期间，应验证该定义并评估其可用性。
