[HTML][HTML] Neonates with cystic fibrosis have a reduced nasal liquid pH; a small pilot study

MH Abou Alaiwa, AM Beer, AA Pezzulo… - Journal of cystic …, 2014 - Elsevier
MH Abou Alaiwa, AM Beer, AA Pezzulo, JL Launspach, RA Horan, DA Stoltz, TD Starner…
Journal of cystic fibrosis, 2014Elsevier
Background Disrupted HCO 3–transport and reduced airway surface liquid (ASL) pH in
cystic fibrosis (CF) may initiate airway disease. We hypothesized that ASL pH is reduced in
neonates with CF. Methods In neonates with and without CF, we measured pH of nasal ASL.
We also measured nasal pH in older children and adults. Results In neonates with CF, nasal
ASL (pH 5.2±0.3) was more acidic than in non-CF neonates (pH 6.4±0.2). In contrast, nasal
pH of CF children and adults was similar to values measured in people without CF …
Background
Disrupted HCO3 transport and reduced airway surface liquid (ASL) pH in cystic fibrosis (CF) may initiate airway disease. We hypothesized that ASL pH is reduced in neonates with CF.
Methods
In neonates with and without CF, we measured pH of nasal ASL. We also measured nasal pH in older children and adults.
Results
In neonates with CF, nasal ASL (pH 5.2 ± 0.3) was more acidic than in non-CF neonates (pH 6.4 ± 0.2). In contrast, nasal pH of CF children and adults was similar to values measured in people without CF.
Conclusions
At an age when infection, inflammation and airway wall remodeling are minimal, neonates with CF had an acidic nasal ASL compared to babies without CF. The CF:non-CF pH difference disappeared in older individuals, perhaps because secondary manifestations of disease increase ASL pH. These results aid understanding of CF pathogenesis and suggest opportunities for therapeutic intervention and monitoring of disease.
Elsevier