Primary ciliary dyskinesia: Overlooked and undertreated in children

Patrick Hosie, Dominic A. Fitzgerald*, Adam Jaffe, Catherine S. Birman, Lucy Morgan

*Corresponding author for this work

    Research output: Contribution to journalReview articlepeer-review

    9 Citations (Scopus)

    Abstract

    Primary ciliary dyskinesia (PCD) is a multi-organ disorder associated with chronic oto-sino-pulmonary disease, neonatal respiratory distress, situs abnormalities and reduced fertility. Repeated respiratory tract infections leads to the almost universal development of bronchiectasis. These clinical manifestations are a consequence of poorly functioning motile cilia. However, confirming the diagnosis is quite difficult and is often delayed, so the true incidence of PCD may be significantly higher than current estimates. Nasal nitric oxide has been earmarked as a useful screening tool for identifying patients, but its use is limited in pre-school-aged children. Due to the rarity of PCD, the evidence base for management is somewhat limited, and treatment regimens are extrapolated from other suppurative lung disorders, like cystic fibrosis.

    Original languageEnglish
    Pages (from-to)952-958
    Number of pages7
    JournalJournal of Paediatrics and Child Health
    Volume50
    Issue number12
    DOIs
    Publication statusPublished - 1 Dec 2014

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