Related Experiment Video
Updated: Oct 2, 2025

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
Pulmonary Exacerbations in Pediatric Patients: Retrospective Study in a Portuguese Cystic Fibrosis Center
Rosa Cardoso1, Ana Lúcia Cardoso2, Telma Barbosa2
1Instituto de Ciências Biomédicas Abel Salazar, Universidade do Porto, 4050-313 Porto, Portugal.
Insights
Pulmonary exacerbations (PEx) in pediatric cystic fibrosis (CF) patients occurred with a median frequency of 1.0 per year. Common symptoms included increased cough and changes in secretions, with favorable outcomes for all PEx.
Area of Science:
- Pediatric Pulmonology
- Genetics
- Infectious Diseases
Background:
- Cystic fibrosis (CF) is a genetic disorder affecting multiple organs, primarily the lungs.
- Pulmonary exacerbations (PEx) represent acute symptom worsening and are a major cause of morbidity and mortality in CF patients.
- Understanding PEx in pediatric populations is crucial for effective disease management.
Purpose of the Study:
- To determine the frequency of PEx in pediatric CF patients.
- To characterize the clinical presentation and microbial causes of PEx.
- To explore associations between PEx characteristics and patient features.
Main Methods:
- Retrospective analysis of PEx data from January 2015 to December 2019.
- Inclusion of pediatric patients from a single CF center.
- Descriptive statistics and chi-square/Fisher's exact test for data analysis.
Main Results:
- 198 PEx were identified in 34 pediatric patients, with a median of 1.0 PEx/patient/year.
- Increased cough (93.9%) and altered secretions (88.4%) were the most frequent symptoms.
- Staphylococcus aureus (54.9%) and Pseudomonas aeruginosa (24.9%) were the predominant pathogens; most PEx were treated outpatient with favorable outcomes.
Conclusions:
- The frequency and characteristics of PEx in this pediatric CF cohort align with existing literature.
- Further research is needed to better characterize CF PEx.
- Standardized management protocols for pediatric CF PEx require further development.
Abstract:
(1) Background: Cystic fibrosis (CF) is a multisystemic disease caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. Lung disease, the leading cause of morbimortality, is marked by acute worsening of symptoms-such as pulmonary exacerbations (PEx). The objectives of this study were: Identifying the frequency of PEx in pediatric CF patients; Characterizing each PEx; Finding association between the frequency and characteristics of the PEx and patients' features. (2) Methods: Retrospective analysis of all PEx from a period of January 2015 to December 2019 in a group of pediatric patients from a single CF center. Data were collected from medical records. Descriptive statistics and chi-square/Fisher's test were used. (3) Results: Thirty-four pediatric patients contributed to the total sample used in this study and 198 PEx were identified, median of 1.0 PEx/patient/year. Most frequent PEx symptoms were increased cough (93.9%) and change in secretions (88.4%), most common pathogens were Staphylococcus aureus (54.9%) and Pseudomonas aeruginosa (24.9%). The majority were treated as outpatient (85.9%). Most common antibiotics included amoxicillin/clavulanate (35.9%) and ciprofloxacin (22.7%). Outcome was favorable in all PEx. (4) Conclusion: Results were consistent with what has been described in literature. More studies are necessary for a better characterization of CF PEx, in order to develop standardized protocols for their management.
Related Concept Videos
Cystic Fibrosis: Management
Sinus disease and chronic...
Chronic Obstructive Pulmonary Disease-IV: Assessement and Diagnostic Studies
Medical History
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,...
Pulmonary Cycle: Exhalation
COPD: Pathogenesis and Clinical Features
The primary cause for the onset of COPD is cigarette smoking and exposure to air pollution. These hazardous factors initiate a chain reaction within the lungs, resulting in chronic inflammation, damage to the airways, and a...
COPD: Management Using Bronchodilators and Corticosteroids

