RT Journal Article SR Electronic T1 Pulmonary exacerbations in patients with primary ciliary dyskinesia: an expert consensus definition for use in clinical trials JF ERJ Open Research JO erjor FD European Respiratory Society SP 00147-2018 DO 10.1183/23120541.00147-2018 VO 5 IS 1 A1 Jane S. Lucas A1 Florian Gahleitner A1 Adelina Amorim A1 Mieke Boon A1 Philippa Brown A1 Carolina Constant A1 Simon Cook A1 Suzanne Crowley A1 Damien M.S. Destouches A1 Ernst Eber A1 Huda Mussaffi A1 Eric Haarman A1 Amanda Harris A1 Cordula Koerner-Rettberg A1 Claudia E. Kuehni A1 Philipp Latzin A1 Michael R. Loebinger A1 Natalie Lorent A1 Bernard Maitre A1 Antonio Moreno-Galdó A1 Kim G. Nielsen A1 Uğur Özçelik A1 Lue Katrine Drasbæk Philipsen A1 Petr Pohunek A1 Eva Polverino A1 Jessica Rademacher A1 Phil Robinson A1 Deborah Snijders A1 Panayiotis Yiallouros A1 Siobhán B. Carr A1 , YR 2019 UL http://openres.ersjournals.com/content/5/1/00147-2018.abstract AB 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