Presenting features in primary ciliary dyskinesia vary with age

EUROPEAN RESPIRATORY JOURNAL(2016)

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摘要
Background: PCD presentation is non-specific and can lead to missed diagnosis. Wet cough, rhinitis, infertility and neonatal respiratory distress (NRD) are considered “classical” in PCD, however there is no data on whether this varies with age. Objectives: To compare presenting features at time of diagnosis in PCD patients of different ages. Methods: Prospectively collected data from 75 patients diagnosed with PCD. Patients were age-grouped ( 35 years) and clinical features recorded. Results: Cough (82-100%) and persistent rhinitis were common in all age groups, though rhinitis was less common in 1 years had a history of glue ear. Significantly more adults than children presented with bronchiectasis (90 v 10%, and sinusitis (60% v 17%, pu003c0.01) Conclusion: Persistent wet cough is a “hallmark” feature of PCD. Other features varied; fewer nasal symptoms in
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Epidemiology
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