Pulmonary Exacerbations in Pediatric Patients: Retrospective Study in a Portuguese Cystic Fibrosis Center

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.

Keywords

• cystic fibrosis
• pulmonary exacerbations
• infection
• lung function