Chest
Volume 145, Issue 4, April 2014, Pages 674-676
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Primary Ciliary Dyskinesia and Cystic Fibrosis: Different Diseases Require Different Treatment

https://doi.org/10.1378/chest.13-2590Get rights and content

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Cited by (22)

  • Primary ciliary dyskinesia in the genomics age

    2020, The Lancet Respiratory Medicine
    Citation Excerpt :

    Management is often extrapolated from other diseases, such as cystic fibrosis and chronic rhinosinusitis. Although both cystic fibrosis and primary ciliary dyskinesia inevitably lead to bronchiectasis, their underlying pathomechanisms and clinical course are different.2,113–115 Response to treatment is likely to vary in primary ciliary dyskinesia and international collaborations are urgently needed to ensure a sufficient number of eligible patients for well designed treatment trials.

  • Fitness and lung function in children with primary ciliary dyskinesia and cystic fibrosis

    2018, Respiratory Medicine
    Citation Excerpt :

    CF treatment is based on a growing number of evidence-based guidelines and clinical trials. In contrast, PCD treatment is mainly based on application of CF guidelines [7], which is justified to some degree by the common reduction in mucociliary clearance, but questionable due to the huge differences in basic pathophysiology. Moreover, PCD is often diagnosed at a substantially higher age than CF [16], which was also apparent in the present study; however, age at diagnosis does not seem to be significantly correlated with pulmonary function in PCD [27,33].

  • Primary ciliary dyskinesia in adults

    2016, Revue des Maladies Respiratoires
    Citation Excerpt :

    The treatment is based on expert opinions, as in many rare diseases [2], and is centered on the pediatric management. Most treatments are proposed by analogy with those used in (idiopathic or secondary) bronchiectasis [45] or in cystic fibrosis [110]. The current treatment is based on chest physiotherapy, antibiotics and avoidance of risky behaviors (active or passive smoking).

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Financial/nonfinancial disclosures: The authors have reported to CHEST that no potential conflicts of interest exist with any companies/organizations whose products or services may be discussed in this article.

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