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

André Schultz (), Ramaa Puvvadi, Sergey M. Borisov, Nicole C. Shaw, Ingo Klimant, Luke J. Berry, Samuel T. Montgomery, Thien Nguyen, Silvia M. Kreda, Anthony Kicic, Peter B. Noble, Brian Button and Stephen M. Stick
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André Schultz: University of Western Australia
Ramaa Puvvadi: University of Western Australia
Sergey M. Borisov: Graz University of Technology
Nicole C. Shaw: University of Western Australia
Ingo Klimant: Graz University of Technology
Luke J. Berry: University of Western Australia
Samuel T. Montgomery: University of Western Australia
Thien Nguyen: University of North Carolina
Silvia M. Kreda: University of North Carolina
Anthony Kicic: University of Western Australia
Peter B. Noble: University of Western Australia
Brian Button: University of North Carolina
Stephen M. Stick: University of Western Australia

Nature Communications, 2017, vol. 8, issue 1, 1-9

Abstract: Abstract 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.

Date: 2017
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DOI: 10.1038/s41467-017-00532-5

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