… lung function and reducing pulmonary exacerbations [4]. … In patients with these three diseases,
the associated … mutation hot spots on DNAI1 and DNAH5 by direct sequencing, but if no …

… clearance leads to chronic mucopurulent airway disease that … in spite of the overall high
involvement of DNAH5, none of its … that PCD lung function outcomes are related to individual …

… the broad spectrum of phenotypes associated with PCD and … disease causing variants leading
to ODA defects (eg DNAH5, … by a mucopurulent airway disease. The estimated incidence …

… symptoms consistent with PCD and benefit from respiratory therapy for airway clearance.
PCD was shown to be associated with prolonged length of stay and decreased lung function …

… DNAH5 mutations).[ 19] In 10-12% of the patients, there is heterotaxy associated with congenital
heart disease… There is heterogeneity in lung function among patients with PCD. Reports …

… -pulmonary disease that can result in early death or need for … disease but severe bronchiectasis
with very low lung function… (B, DNAH5), the ODA heavy chain DNAH5 is absent from the …

… Chronic respiratory symptoms associated with PCD can … with cystic fibrosis and chronic
obstructive pulmonary disease.… variants in DNAH5 may explain some of the increased respiratory …

… In six patients with VUS in CCDC40, CCDC103, DNAH5, and … , was presumably associated
with normal ultrastructure. … PCD had chronic upper airway disease, chronic wet cough, …

… Pathogenic variants of DNAH5 are associated with primary ciliary dyskinesia (PCD) with …
could cause interstitial pulmonary fibrosis. Pathogenic variants in LTBP4 are associated with “…

… associated with severe disease related to cardiovascular … Arg3539His in DNAH5 were
detected in two patients with a … -19 patients with chronic obstructive pulmonary disease, cystic …