TY - JOUR T1 - Pulmonary exacerbations in patients with primary ciliary dyskinesia: an expert consensus definition for use in clinical trials JF - ERJ Open Research JO - erjor DO - 10.1183/23120541.00147-2018 VL - 5 IS - 1 SP - 00147-2018 AU - Jane S. Lucas AU - Florian Gahleitner AU - Adelina Amorim AU - Mieke Boon AU - Philippa Brown AU - Carolina Constant AU - Simon Cook AU - Suzanne Crowley AU - Damien M.S. Destouches AU - Ernst Eber AU - Huda Mussaffi AU - Eric Haarman AU - Amanda Harris AU - Cordula Koerner-Rettberg AU - Claudia E. Kuehni AU - Philipp Latzin AU - Michael R. Loebinger AU - Natalie Lorent AU - Bernard Maitre AU - Antonio Moreno-Galdó AU - Kim G. Nielsen AU - Uğur Özçelik AU - Lue Katrine Drasbæk Philipsen AU - Petr Pohunek AU - Eva Polverino AU - Jessica Rademacher AU - Phil Robinson AU - Deborah Snijders AU - Panayiotis Yiallouros AU - Siobhán B. Carr A2 - , Y1 - 2019/02/01 UR - http://openres.ersjournals.com/content/5/1/00147-2018.abstract N2 - 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.A consensus definition for pulmonary exacerbations in children and adults with PCD for use in clinical trials http://ow.ly/Rcfr30n4Gn4 ER -