Research
Print page Print page
Switch language
The Capital Region of Denmark - a part of Copenhagen University Hospital
Published

Clinical care of children with primary ciliary dyskinesia

Research output: Contribution to journalJournal articleResearchpeer-review

  1. Is there a link between female asthma and a reduction in fertility?

    Research output: Contribution to journalJournal articleResearchpeer-review

  2. The asthma-plus syndrome

    Research output: Contribution to journalJournal articleResearchpeer-review

  3. The usefulness of the mannitol challenge test for asthma

    Research output: Contribution to journalJournal articleResearchpeer-review

  4. Elite athletes with asthma: is the management goal total control?

    Research output: Contribution to journalJournal articleResearchpeer-review

  5. Asthma in elite athletes

    Research output: Contribution to journalJournal articleResearchpeer-review

  1. SPEF2- And HYDIN-mutant cilia lack the central pair-associated protein SPEF2, aiding primary ciliary dyskinesia diagnostics

    Research output: Contribution to journalJournal articleResearchpeer-review

  2. Ciliary Localization of the Intraflagellar Transport Protein IFT88 Is Disrupted in Cystic Fibrosis

    Research output: Contribution to journalJournal articleResearchpeer-review

  3. Primary ciliary dyskinesia patients have the same P. aeruginosa clone in sinuses and lungs

    Research output: Contribution to journalJournal articleResearchpeer-review

  4. The Controversies and Difficulties of Diagnosing Primary Ciliary Dyskinesia

    Research output: Contribution to journalJournal articleResearchpeer-review

View graph of relations

INTRODUCTION: Primary ciliary dyskinesia (PCD) is a rare heterogeneous disorder, usually inherited as an autosomal recessive condition but X-linked inheritance is also described. Abnormal ciliary function in childhood leads to neonatal respiratory distress in term infants, persistent wet cough, bronchiectasis, chronic rhinosinusitis, and hearing impairment; approximately 50% of patients have situs inversus. There is a paucity of evidence for treating PCD, hence consensus guidelines are predominantly influenced by knowledge from cystic fibrosis (CF). Extrapolation of evidence from other diseases is inappropriate since differences in pathophysiology, morbidity and prognosis risk treatment failure and lack of adherence. Areas covered: Review authors searched PubMed and Cochrane databases for publications relating to management of children with PCD. Because of the paucity of data, we emphasise the need for well-designed clinical trials with PCD patients rather than reliance on evidence from other diseases. Expert commentary: The evidence for treatment of PCD is poor, and management is often extrapolated from studies of patients with CF or chronic rhinosinusitis. However, much work is underway to improve the situation and international consortia and networks are conducting well-designed projects to inform the management of children with PCD.

Original languageEnglish
JournalExpert Review of Respiratory Medicine
Volume11
Issue number10
Pages (from-to)779-790
Number of pages12
ISSN1747-6348
DOIs
Publication statusPublished - Oct 2017

    Research areas

  • Journal Article

ID: 52618433