Table 1– Recommendations for diagnosis and management of primary ciliary dyskinesia (PCD)
A) Centralised care
 1) Patients with PCD should be seen for either full or shared care in a centre specialising in the condition
B) Diagnosis
 2) Nasal NO levels can be used as a screening test for PCD in children, while the saccharine test is unreliable
 3) Diagnosis is confirmed by analysis of ciliated epithelial cells derived from nasal brushings or bronchoscopic samples
 4) Ciliary beat pattern and frequency analyses using high-speed video recording and electron microscopy are the key diagnostic techniques; other techniques (cell culture, analysis of dynein protein localisation by immunofluorescence and genetic analyses) might help in selected patients
C) Treatment
 5) Airway clearance by physiotherapy and exercise, and prompt antibiotic treatment (oral, intravenous if needed) are the cornerstones of treatment
 6) Prophylactic oral antibiotics and long-term use of nebulised anti-Pseudomonas antibiotics should be considered in specific patients
 7) Inhaled bronchodilators and topical or inhaled steroids have no routine place in PCD treatment except for patients with concurrent asthma; rhDNase and hypertonic saline might possibly be considered in very selected patients
 8) The use of tympanostomic ventilation tubes should be avoided for PCD patients whenever possible
 9) All PCD patients should receive all childhood immunisations including pneumococcal and influenza immunisation
D) Follow-up
 10) A protocolised shared-care system is recommended to ensure specialist follow-up and prevent eventual lung damage; regular sputum or cough-swab cultures should be performed
  • NO: nitric oxide; rhDNase: recombinant human deoxyribonuclease. Summarised from the consensus statement of the European Respiratory Society Task Force on PCD in children [7].