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 |