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    Airway surface liquid pH is not acidic in children with cystic fibrosis

    Access Status
    Fulltext not available
    Authors
    Schultz, A.
    Puvvadi, R.
    Borisov, S.
    Shaw, N.
    Klimant, I.
    Berry, L.
    Montgomery, S.
    Nguyen, T.
    Kreda, S.
    Kicic, Anthony
    Noble, P.
    Button, B.
    Stick, S.
    Date
    2017
    Type
    Journal Article
    
    Metadata
    Show full item record
    Citation
    Schultz, A. and Puvvadi, R. and Borisov, S. and Shaw, N. and Klimant, I. and Berry, L. and Montgomery, S. et al. 2017. Airway surface liquid pH is not acidic in children with cystic fibrosis. Nature Communications. 8 (1).
    Source Title
    Nature Communications
    DOI
    10.1038/s41467-017-00532-5
    ISSN
    2041-1723
    School
    School of Public Health
    URI
    http://hdl.handle.net/20.500.11937/72846
    Collection
    • Curtin Research Publications
    Abstract

    © 2017 The Author(s). Modulation of airway surface liquid (ASL) pH has been proposed as a therapy for cystic fibrosis (CF). However, evidence that ASL pH is reduced in CF is limited and conflicting. The technical challenges associated with measuring ASL pH in vivo have precluded accurate measurements in humans. In order to address this deficiency, ASL pH was measured in vivo in children using a novel luminescent technology integrated with fibre-optic probes. Here we show that ASL pH in children with CF is similar to that of children without CF. Findings were supported by highly controlled direct pH measurements in primary human airway epithelial cell culture models, which also suggest that the potential ASL pH gradient produced by defective apical ion transport is balanced out by paracellular shunting of acid/base. Thus, reduced baseline ASL pH is unlikely to be an important pathobiological factor in early CF lung disease.

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