Management of primary ciliary dyskinesia: the lower airways

Paediatr Respir Rev. 2009 Jun;10(2):55-7. doi: 10.1016/j.prrv.2008.10.005. Epub 2008 Nov 28.


Primary ciliary dyskinesia (PCD) is a genetic disease associated with defective ciliary structure and function. Chronic oto-sino-pulmonary infection is the most common clinical presentation. Patients should be monitored in centres with expertise in PCD. Regular respiratory monitoring consists of lung function tests, adapted to the patient's age, and cough swab or sputum cultures. Chest X-rays are insensitive but the use of high-resolution computed tomography should be considered carefully to avoid excessive radiation. Treatment of the chronic suppuration of the lower airways relies on antibiotics to which the isolated bacteria are sensitive, together with respiratory physiotherapy and exercise to clear excessive bronchial secretions. Bronchodilators and anti-inflammatory agents have no proven benefit and should be prescribed, if at all, on an individual basis. Treatment of the lower airways should always be in conjunction with that of the upper airways. Importantly in PCD, respiratory disease may be controlled with the use of early, adequate and aggressive management.

Publication types

  • Review

MeSH terms

  • Anti-Bacterial Agents / therapeutic use*
  • Diagnostic Imaging
  • Humans
  • Kartagener Syndrome / complications
  • Kartagener Syndrome / diagnosis
  • Kartagener Syndrome / therapy*
  • Physical Therapy Modalities*
  • Respiratory Tract Infections / diagnosis
  • Respiratory Tract Infections / etiology
  • Respiratory Tract Infections / prevention & control*
  • Treatment Outcome


  • Anti-Bacterial Agents