HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS		QUICK SEARCH:   [advanced] Author: Keyword(s): Year:  Vol:  Page:

This Article Full Text Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Permissions Request Permissions Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via HighWire Google Scholar Articles by Barbato, A. Articles by Bush, A. PubMed PubMed Citation Articles by Barbato, A. Articles by Bush, A.

Primary ciliary dyskinesia: a consensus statement on diagnostic and treatment approaches in children

¹ For affiliations, please see the Acknowledgements section, and ² All of the authors of this article were active task force members.

CORRESPONDENCE: A. Barbato, Paediatric Pulmonology Unit, Dept of Paediatrics, Via Giustiniani 3, 35128 Padova, Italy. E-mail: barbato{at}pediatria.unipd.it

Keywords: Consensus statement, diagnosis, primary ciliary dyskinesia, treatment

Received: November 21, 2008
Accepted May 12, 2009

Primary ciliary dyskinesia (PCD) is associated with abnormal ciliary structure and function, which results in retention of mucus and bacteria in the respiratory tract, leading to chronic oto-sino-pulmonary disease, situs abnormalities and abnormal sperm motility.

The diagnosis of PCD requires the presence of the characteristic clinical phenotype and either specific ultrastructural ciliary defects identified by transmission electron microscopy or evidence of abnormal ciliary function.

Although the management of children affected with PCD remains uncertain and evidence is limited, it remains important to follow-up these patients with an adequate and shared care system in order to prevent future lung damage.

This European Respiratory Society consensus statement on the management of children with PCD formulates recommendations regarding diagnostic and therapeutic approaches in order to permit a more accurate approach in these patients. Large well-designed randomised controlled trials, with clear description of patients, are required in order to improve these recommendations on diagnostic and treatment approaches in this disease.

This article has been cited by other articles:

				W. A. Stannard, M. A. Chilvers, A. R. Rutman, C. D. Williams, and C. O'Callaghan Diagnostic Testing of Patients Suspected of Primary Ciliary Dyskinesia Am. J. Respir. Crit. Care Med., February 15, 2010; 181(4): 307 - 314. [Abstract] [Full Text] [PDF]