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

Nat Commun. 2017 Nov 10;8(1):1409. doi: 10.1038/s41467-017-00532-5.

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.

Publication types

  • Observational Study
  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Bacterial Infections / complications
  • Bacterial Infections / metabolism
  • Bronchi / metabolism
  • Bronchoalveolar Lavage Fluid / chemistry
  • Bronchoalveolar Lavage Fluid / microbiology
  • Cells, Cultured
  • Child
  • Child, Preschool
  • Cystic Fibrosis / complications
  • Cystic Fibrosis / etiology
  • Cystic Fibrosis / metabolism*
  • Female
  • Fiber Optic Technology
  • Fluorescent Dyes
  • H(+)-K(+)-Exchanging ATPase / metabolism
  • Humans
  • Hydrogen-Ion Concentration
  • Infant
  • Male
  • Prospective Studies
  • Respiratory Mucosa / metabolism*

Substances

  • Fluorescent Dyes
  • ATP12A protein, human
  • H(+)-K(+)-Exchanging ATPase