Chest
EditorialsPrimary Ciliary Dyskinesia and Cystic Fibrosis: Different Diseases Require Different Treatment
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Cited by (22)
Cystic fibrosis and primary ciliary dyskinesia: Similarities and differences
2023, Respiratory MedicinePrimary ciliary dyskinesia in the genomics age
2020, The Lancet Respiratory MedicineCitation 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.
Primary ciliary dyskinesia among Arabs: Where do we go from here?
2019, Paediatric Respiratory ReviewsFitness and lung function in children with primary ciliary dyskinesia and cystic fibrosis
2018, Respiratory MedicineCitation 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].
Airway Clearance Techniques for Primary Ciliary Dyskinesia; is the Cystic Fibrosis literature portable?
2018, Paediatric Respiratory ReviewsPrimary ciliary dyskinesia in adults
2016, Revue des Maladies RespiratoiresCitation 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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