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Neonates with cystic fibrosis have a reduced nasal liquid pH; a small pilot study.


ABSTRACT: 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.In neonates with and without CF, we measured pH of nasal ASL. We also measured nasal pH in older children and adults.In neonates with CF, nasal ASL (pH5.2 ± 0.3) was more acidic than in non-CF neonates (pH6.4 ± 0.2). In contrast, nasal pH of CF children and adults was similar to values measured in people without CF.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.

SUBMITTER: Abou Alaiwa MH 

PROVIDER: S-EPMC4060428 | biostudies-literature | 2014 Jul

REPOSITORIES: biostudies-literature

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Neonates with cystic fibrosis have a reduced nasal liquid pH; a small pilot study.

Abou Alaiwa Mahmoud H MH   Beer Alison M AM   Pezzulo Alejandro A AA   Launspach Janice L JL   Horan Rebecca A RA   Stoltz David A DA   Starner Timothy D TD   Welsh Michael J MJ   Zabner Joseph J  

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society 20140111 4


<h4>Background</h4>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.<h4>Methods</h4>In neonates with and without CF, we measured pH of nasal ASL. We also measured nasal pH in older children and adults.<h4>Results</h4>In neonates with CF, nasal ASL (pH5.2 ± 0.3) was more acidic than in non-CF neonates (pH6.4 ± 0.2). In contrast, nasal pH of CF children and adults w  ...[more]

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