INTRODUCTION: In primary ciliary dyskinesia (PCD) impaired mucociliary clearance leads to recurrent airway infections and progressive lung destruction, and concern over chronic airway infection and patient-to-patient transmission is considerable. So far, there has been no defined consensus on how to control infection across centres caring for patients with PCD. Within the BEAT-PCD network, COST Action and ERS CRC together with the ERN-Lung PCD core a first initiative has now been taken towards creating such a consensus statement. METHODS: A multidisciplinary international PCD expert panel was set up to create a consensus statement for infection prevention and control (IP&C) for PCD, covering diagnostic microbiology, infection prevention for specific pathogens considered indicated for treatment and segregation aspects. Using a modified Delphi process, consensus to a statement demanded at least 80% agreement within the PCD expert panel group. Patient organisation representatives were involved throughout the process. RESULTS: We present a consensus statement on 20 IP&C statements for PCD including suggested actions for microbiological identification, indications for treatment of Pseudomonas aeruginosa, Burkholderia cepacia and nontuberculous mycobacteria and suggested segregation aspects aimed to minimise patient-to-patient transmission of infections whether in-hospital, in PCD clinics or wards, or out of hospital at meetings between people with PCD. The statement also includes segregation aspects adapted to the current coronavirus disease 2019 (COVID-19) pandemic. CONCLUSION: The first ever international consensus statement on IP&C intended specifically for PCD is presented and is targeted at clinicians managing paediatric and adult patients with PCD, microbiologists, patient organisations and not least the patients and their families.

Association des Patients Ayant une Dyskinésie Ciliaire Primitive Limeil Brevannes France

CIBER of Rare Diseases Madrid Spain

Danish PCD Centre Copenhagen Paediatric Pulmonary Service Copenhagen University Hospital Rigshospitalet Copenhagen Denmark

Dept of Clinical Medicine University of Copenhagen Copenhagen Denmark

Dept of Clinical Microbiology University Hospital Rigshospitalet Copenhagen Denmark

Dept of General Paediatrics and Adolescent Medicine University Hospital Muenster Muenster Germany

Dept of Paediatrics University Hospital Gasthuisberg Leuven Belgium

Dept of Paediatrics University of Melbourne Parkville Australia

Dept of Pediatric Pulmonology VU University Medical Center Amsterdam The Netherlands

Dept of Pediatrics Vall d'Hebron Hospital Universitari Vall d'Hebron Barcelona Hospital Campus Universitat Autònoma de Barcelona Barcelona Spain

Dept of Respiratory and Sleep Medicine Royal Children's Hospital Parkville Australia

Depts of Paediatrics and Paediatric Respiratory Medicine Imperial College and Royal Brompton Hospital London UK

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

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

Kartagener Syndrom und Primäre Ciliäre Dyskinesie Steffisburg Switzerland

Medical School University of Cyprus Nicosia Cyprus

Murdoch Children's Research Institute Parkville Australia

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

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

Patient representative

Pediatric Pulmonology Unit Hospital Clínico Universitario de Valencia University of Valencia Valencia Spain

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

Primary Ciliary Dyskinesia Centre NIHR Respiratory Biomedical Research Centre Clinical and Experimental Science University of Southampton Southampton UK

Pulmonary Service Centre constitutif Respirare Centre Hospitalier intercommunal de Créteil Univ Paris Est Creteil INSERM IMRB Creteil France

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

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Associations between respiratory pathogens and lung function in primary ciliary dyskinesia: cross-sectional analysis from the PROVALF-PCD cohort