The DOI matches its Crossref record; 60 of the 60 references it cites were found in a registry or an index.
Airway Surface Dehydration in Cystic Fibrosis: Pathogenesis and Therapy
Abstract
Cystic fibrosis (CF) lung disease reflects the failure of airways defense against chronic bacterial infection. Studies of CF cultures, transgenic mice, and CF patients suggest that the initiating event in CF airways disease pathogenesis is reduced airway surface liquid (ASL) volume, i.e., dehydration. CF ASL volume regulation depends on a single extracellular signaling system, ATP, which renders CF airways more vulnerable to disease-causing insults (e.g., viruses) than are normal airways, which regulate ASL volume by dual ATP and adenosine signaling pathways. Clinical studies have explored the hypothesis that treating the dehydration of CF airways will be therapeutically beneficial. Inhaled hypertonic saline osmotically draws water onto airway surfaces, improves mucus clearance and pulmonary function, and reduces acute exacerbations in CF patients. Thus, rehydration therapies may slow the progression of CF lung disease in patients with established bacterial infection and may prevent the onset of CF lung disease if initiated early in life.
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Cites
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Self-citationMucus clearance as a primary innate defense mechanism for mammalian airways
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Self-citationCystic Fibrosis Airway Epithelia Fail to Kill Bacteria Because of Abnormal Airway Surface Fluid
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Self-citationLubrication by charged polymers
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A Controlled Trial of Long-Term Inhaled Hypertonic Saline in Patients with Cystic Fibrosis
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Cited by
O'Sullivan, B. P., & Freedman, S. D. (2009) · The Lancet · doi:10.1016/s0140-6736(09)60327-5 · Cited by 1,320
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The innate immune function of airway epithelial cells in inflammatory lung disease
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Cystic fibrosis: impaired bicarbonate secretion and mucoviscidosis
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Cystic fibrosis airway secretions exhibit mucin hyperconcentration and increased osmotic pressure
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Metadata
- Authors
- Boucher, R. C.
- Venue
- Annual Review of Medicine
- Publisher
- Annual Reviews
- Published
- 2007-02-01
- Volume
- 58
- Issue
- 1
- Pages
- 157-170
- ISSN
- 0066-4219 (ISSN-L)
- 1545-326X
- OpenAlex
- W2133653432
- Cited by
- 512
- References
- 60
- FWCI
- 4.15
- Type
- journal article
- Language
- en
- Open access
- closed
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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).
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