Related Experiment Video
Updated: May 13, 2026

The WinCF Model - An Inexpensive and Tractable Microcosm of a Mucus Plugged Bronchiole to Study the Microbiology of Lung Infections
Published on: May 8, 2017
Respiratory bacterial infections in cystic fibrosis
Oana Ciofu1, Christine R Hansen, Niels Høiby
1Institute of International Health, Immunology and Microbiology, Unit of Bacteriology, Faculty of Health and Medical Sciences, University of Copenhagen, Copenhagen, Denmark. ociofu@sund.ku.dk
Purpose Of Review:
Bacterial respiratory infections are the main cause of morbidity and mortality in patients with cystic fibrosis (CF). Pseudomonas aeruginosa remains the main pathogen in adults, but other Gram-negative bacteria such as Achromobacter xylosoxidans and Stenotrophomonas maltophilia as well as nontuberculous mycobacteria have been shown to play an important role in the lung disease. The purpose of this review is to summarize the knowledge on disease and treatment of infection with CF-related pathogens.
Recent Findings:
The role of the paranasal sinuses as a source of infection and site of bacterial adaptation has been recognized. This review will focus on the different conditions encountered by the bacteria in sinuses and lung, as well as the principles of treatment in the different infection sites.
Summary:
Chronic, pulmonary infections remain the single most prominent cause of the increased morbidity and mortality in CF. After the increasing efficiency of anti-P. aeruginosa treatment, newer pathogens have been identified, with individual clinical characteristics. Microbiological surveillance is very important in keeping the patients stable. Samples from both the lower and upper respiratory tract (nasal sampling) should be investigated and both infection sites should be treated.
Insights
Cystic fibrosis (CF) patients face significant risks from bacterial respiratory infections. This review covers the disease and treatment of CF-related pathogens, emphasizing upper and lower respiratory tract surveillance and treatment.
Area of Science:
- Pulmonology
- Infectious Diseases
- Microbiology
Background:
- Bacterial respiratory infections are a primary cause of morbidity and mortality in cystic fibrosis (CF) patients.
- Pseudomonas aeruginosa is a key pathogen in adults, but other bacteria like Achromobacter xylosoxidans, Stenotrophomonas maltophilia, and nontuberculous mycobacteria also contribute to lung disease.
Purpose of the Study:
- To review current knowledge on the disease and treatment of infections caused by CF-related pathogens.
- To discuss bacterial adaptation in the paranasal sinuses and lungs and treatment principles for these sites.
Main Methods:
- Literature review summarizing existing research on CF-related respiratory infections.
- Focus on the role of paranasal sinuses as a reservoir for bacterial adaptation and infection.
- Analysis of treatment principles for infections in both upper (sinuses) and lower (lungs) respiratory tracts.
Main Results:
- Chronic pulmonary infections remain the leading cause of morbidity and mortality in CF.
- Emerging pathogens beyond P. aeruginosa have been identified, each with unique clinical characteristics.
- The paranasal sinuses are recognized as a significant source of infection and bacterial adaptation in CF patients.
Conclusions:
- Effective microbiological surveillance, including nasal sampling, is crucial for patient stability.
- Comprehensive treatment should address infections in both the lower and upper respiratory tracts.
- Understanding and treating diverse CF-related pathogens is essential for improving patient outcomes.
Related Concept Videos
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...
Microbiota of the Respiratory Tract
Cystic Fibrosis: Management
Sinus disease and chronic sinusitis...
Chronic Obstructive Pulmonary Disease III: Chronic Bronchitis Features
Pneumonia I: Introduction
Pneumonia I: Introduction
Risk Factors
Various factors influence the likelihood of developing pneumonia. Age plays a crucial role, with infants, children under two, and individuals over 65 at increased risk due to their...

