Related Experiment Video
Updated: Jul 23, 2026

Generation of Human Nasal Epithelial Cell Spheroids for Individualized Cystic Fibrosis Transmembrane Conductance Regulator Study
Published on: April 11, 2018
Airway function in infants newly diagnosed with cystic fibrosis
Insights
Infants with cystic fibrosis show reduced lung function soon after diagnosis, even without prior respiratory illness. This suggests early airway issues in cystic fibrosis infants, prompting further research into their development.
Area of Science:
- Pediatric Pulmonology
- Genetic Disorders Research
- Infant Health
Background:
- Assesses early lung function in infants diagnosed with cystic fibrosis (CF).
- Investigates if reduced airway function in CF infants is independent of lower respiratory illness.
- Compares airway function between 33 CF infants and 87 healthy controls, adjusting for key covariates.
Discussion:
- Airway function is significantly impaired in infants with CF.
- This impairment is evident even in CF infants without a history of clinically recognized lower respiratory illness.
- Highlights the need to understand the early onset and progression of airway dysfunction in CF.
Key Insights:
- Early diagnosis of cystic fibrosis in infants correlates with reduced lung function.
- Lower respiratory illness is not a prerequisite for early airway dysfunction in CF.
- Findings challenge existing assumptions about the timeline of CF-related lung damage.
Outlook:
- Further research is needed to elucidate the precise mechanisms and natural history of impaired airway function in infants with CF.
- This study may inform earlier therapeutic interventions for cystic fibrosis.
- Emphasizes the importance of comprehensive infant screening for genetic lung diseases.
Abstract:
The lung function of infants with cystic fibrosis is often reduced shortly after diagnosis. We measured the airway function of newly diagnosed infants to test whether this reduction is independent of clinically recognised lower respiratory illness. We compared the airway function of 33 infants with cystic fibrosis and 87 healthy controls after adjustment for sex, age, bodyweight and length, and exposure to maternal smoking. Airway function was significantly reduced in children with cystic fibrosis, even in those without clinically recognised previous lower respiratory illness. Our findings raise important questions about the onset and natural history of impaired airway function in infants with cystic fibrosis.
Related Concept Videos
Breathing
Assessment of Airway, Skin Color, and Use of Accessory Muscles
Introduction
The initial evaluation of a patient's respiratory system...
Cystic Fibrosis: Pathogenesis
CF is primarily caused by a genetic mutation in a chromosome 7 gene coding for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. The most common gene mutation leading to CF is the ΔF508 mutation, but...
Cystic Fibrosis: Management
Sinus disease and chronic sinusitis...
The Bronchial Tree
The trachea, commonly known as the windpipe, is a tube that connects the larynx (voice box) to the bronchi. At a point called the carina, it bifurcates into two primary bronchi. The right primary bronchus is wider, shorter, and more vertical than the left primary...
Cardiopulmonary Resuscitation V: Advanced Airway Management Techniques

