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.

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