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J Cyst Fibros. 2014 Jul;13(4):373-7. doi: 10.1016/j.jcf.2013.12.006. Epub 2014 Jan 11.

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

Author information

1
Department of Internal Medicine, Roy J. and Lucille A. Carver College of Medicine, University of Iowa, Iowa City, IA 52242, USA.
2
Department of Pediatrics, Roy J. and Lucille A. Carver College of Medicine, University of Iowa, Iowa City, IA 52242, USA.
3
Department of Internal Medicine, Roy J. and Lucille A. Carver College of Medicine, University of Iowa, Iowa City, IA 52242, USA; Howard Hughes Medical Institute, Roy J. and Lucille A. Carver College of Medicine, University of Iowa, Iowa City, IA 52242, USA; Department of Molecular Physiology and Biophysics, Roy J. and Lucille A. Carver College of Medicine, University of Iowa, Iowa City, IA 52242, USA. Electronic address: michael-welsh@uiowa.edu.
4
Department of Internal Medicine, Roy J. and Lucille A. Carver College of Medicine, University of Iowa, Iowa City, IA 52242, USA. Electronic address: joseph-zabner@uiowa.edu.

Abstract

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 (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.

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.

KEYWORDS:

Airway surface liquid (ASL); Cystic fibrosis; Neonatal screen; Neonates; pH

PMID:
24418186
PMCID:
PMC4060428
DOI:
10.1016/j.jcf.2013.12.006
[Indexed for MEDLINE]
Free PMC Article

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