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.

Association des Patients Ayant une Dyskinésie Ciliaire Primitive France

Danish PCD and chILD Centre CF Centre Copenhagen Paediatric Pulmonary Service ERN Accredited for PCD and CF Health Care Dept of Paediatrics and Adolescent Medicine Copenhagen University Hospital Rigshospitalet Copenhagen Denmark

Dept of Paediatric Pneumology University Children's Hospital of Ruhr University Bochum Germany

Dept of Paediatrics University Hospital Gasthuisberg Leuven Belgium

Dept of Pediatric Pulmonology University Medical Faculty Ankara Turkey

Dept of Pediatric Pulmonology VU University Medical Center Amsterdam The Netherlands

Dept of Respiratory Medicine Hannover Medical School Hanover Germany

Dept of Respiratory Medicine University Hospitals Leuven Leuven Belgium

Division of Paediatric Pulmonology and Allergology Dept of Paediatrics and Adolescent Medicine Medical University of Graz Graz Austria

Faculty of Medicine Porto University Porto Portugal

Host Defence Unit Royal Brompton and Harefield NHS Foundation Trust London UK

Imperial College London London UK

Institute of Social and Preventive Medicine University of Bern Bern Switzerland

Medical School University of Cyprus Nicosia Cyprus

Paediatric Dept 2nd Faculty of Medicine Charles University and Motol University Hospital Prague Czech Republic

Paediatric Dept of Allergy and Lung Diseases Oslo University Hospital Oslo Norway

Paediatric Pulmonology Unit Paediatrics Dept Centro Hospitalar Lisboa Norte Lisbon Academic Medical Centre Lisbon Portugal

Paediatric Respiratory Medicine University Children's Hospital of Bern University of Bern Bern Switzerland

PCD Family Support Group UK

PCD Service Dept of Respiratory and Sleep Medicine Royal Children's Hospital Melbourne Australia

Primary Ciliary Dyskinesia Centre Dept of Paediatric Respiratory Medicine Imperial College and Royal Brompton Hospital London UK

Primary Ciliary Dyskinesia Centre Dept of Woman and Child Health University of Padova Padua Italy

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

Schneider Children's Medical Center of Israel Petach Tikva and Sackler School of Medicine Tel Aviv University Tel Aviv Israel

Section of Pediatric Allergy Pulmonology and Cystic Fibrosis Hospital Vall d'Hebron Universitat Autónoma de Barcelona CIBERER ISCIII Barcelona Spain

Service de Pneumologie Hôpital Intercommunal de Créteil DHU A TVB Inserm U955 Université Paris Est Créteil Créteil France

Vall d'Hebron Research Institute Respiratory Disease Dept Hospital Vall d'Hebron CIBER Barcelona Spain

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Azithromycin promotes proliferation, and inhibits inflammation in nasal epithelial cells in primary ciliary dyskinesia

International BEAT-PCD consensus statement for infection prevention and control for primary ciliary dyskinesia in collaboration with ERN-LUNG PCD Core Network and patient representatives