The DOI matches its Crossref record; 1 of the 1 references it cites were found in a registry or an index.
Evidence for airway surface dehydration as the initiating event in CF airway disease
Abstract
Abstract.: Cystic fibrosis (CF) lung disease reflects persistent bacterial infection of airway lumens. Several hypotheses have been advanced to link mutations in the CFTR gene to the failure of the CF lung to defend itself against bacterial infection. Amongst the most productive hypotheses at present is the ‘‘low airway surface liquid (ASL) volume’’ or ‘‘dehydration’’ hypothesis. This hypothesis predicts that airway surface dehydration produces the mucus adhesion, inflammation, and bacterial biofilm formation characteristic of CF. Clinical trials of inhaled hypertonic saline have demonstrated therapeutic benefit of manoeuvres designed to rehydrate CF airway surfaces.
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Citations
Cites
Mucus clearance as a primary innate defense mechanism for mammalian airways
Knowles, M. R., & Boucher, R. C. (2002) · Journal of Clinical Investigation · doi:10.1172/jci0215217 · Cited by 1,054
Self-citation
Cited by
CFTR Function and Prospects for Therapy
Riordan, J. R. (2008) · Annual Review of Biochemistry · doi:10.1146/annurev.biochem.75.103004.142532 · Cited by 610
Physicochemical properties of mucus and their impact on transmucosal drug delivery
Leal, J., Smyth, H. D. C., & Ghosh, D. (2017) · International Journal of Pharmaceutics · doi:10.1016/j.ijpharm.2017.09.018 · Cited by 560
A tale of two sites: how inflammation can reshape the microbiomes of the gut and lungs
Scales, B. S., Dickson, R. P., & Huffnagle, G. B. (2016) · Journal of Leukocyte Biology · doi:10.1189/jlb.3mr0316-106r · Cited by 109
Brushed nasal epithelial cells are a surrogate for bronchial epithelial CFTR studies
Brewington, J. J., Filbrandt, E. T., LaRosa, F. J., Moncivaiz, J. D., Ostmann, A. J., Strecker, L. M., & Clancy, J. P. (2018) · JCI Insight · doi:10.1172/jci.insight.99385 · Cited by 106
Suk, J. S., Boylan, N. J., Trehan, K., Tang, B. C., Schneider, C. S., Lin, J.-M. G., Boyle, M. P., Zeitlin, P. L., Lai, S. K., Cooper, M. J., et al. (2011) · Molecular Therapy · doi:10.1038/mt.2011.160 · Cited by 94
Metadata
- Authors
- Boucher, R. C.
- Venue
- Journal of Internal Medicine
- Publisher
- Wiley
- Published
- 2007-01
- Volume
- 261
- Issue
- 1
- Pages
- 5-16
- ISSN
- 0954-6820 (ISSN-L)
- 1365-2796
- OpenAlex
- W2094703380
- Cited by
- 356
- References
- 1
- FWCI
- 19.53
- Type
- journal article
- Language
- en
- Open access
- closed
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- Boucher, R. C. (2007). Evidence for airway surface dehydration as the initiating event in CF airway disease. Journal of Internal Medicine. https://doi.org/10.1111/j.1365-2796.2006.01744.x
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Bibliographic data: Semantic Scholar, OpenAlex, Crossref.
Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).
Semantic Scholar’s data is described in: Kinney, R., et al. (2023). The Semantic Scholar Open Data Platform. arXiv:2301.10140.
