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J Cyst Fibros. 2018 Jan;17(1):105-108. doi: 10.1016/j.jcf.2017.05.005. Epub 2017 Jul 14.

Lower exhaled nitric oxide in infants with Cystic Fibrosis compared to healthy controls.

Author information

1
Paediatric Respiratory Medicine, Department of Pediatrics, Inselspital, Bern University Hospital, University of Bern, Switzerland; Graduate School for Cellular and Biomedical Sciences, University of Bern, Switzerland; University Children's Hospital (UKBB), Basel, Switzerland.
2
Paediatric Respiratory Medicine, Department of Pediatrics, Inselspital, Bern University Hospital, University of Bern, Switzerland.
3
Paediatric Respiratory Medicine, Department of Pediatrics, Inselspital, Bern University Hospital, University of Bern, Switzerland; Division of Respiratory Medicine, University Children's Hospital Zurich, Switzerland.
4
Department of Paediatrics, Respiratory Unit, Lausanne University Hospital, Lausanne, Switzerland.
5
Division of Respiratory Medicine, Children's Hospital Aarau, Switzerland.
6
Paediatric Respiratory Medicine, Department of Pediatrics, Inselspital, Bern University Hospital, University of Bern, Switzerland; University Children's Hospital (UKBB), Basel, Switzerland.
7
Division of Respiratory Medicine, University Children's Hospital Zurich, Switzerland.
8
University Children's Hospital (UKBB), Basel, Switzerland.
9
Paediatric Respiratory Medicine, Department of Pediatrics, Inselspital, Bern University Hospital, University of Bern, Switzerland. Electronic address: carmen.casaulta@insel.ch.

Abstract

Exhaled nitric oxide (FENO) is a well-known, non-invasive airway biomarker. In patients with Cystic Fibrosis (CF) FENO is decreased. To understand if reduced FENO is primary related to Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) dysfunction or an epiphenomenon of chronic inflammation, we measured FENO in 34 infants with CF prior to clinical symptoms and in 68 healthy controls. FENO was lower in CF compared to controls (p=0.0006) and the effect was more pronounced in CF infants without residual CFTR function (p<0.0001). This suggests that FENO is reduced in CF early in life, possibly associated with underlying CFTR dysfunction.

PMID:
28716479
DOI:
10.1016/j.jcf.2017.05.005

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