Clinical factors associated with pulmonary and extrapulmonary complications in pa-tients with cystic fibrosis A single-center observational study

Abstract

Introduction: Cystic fibrosis (CF) affects the exocrine epithelium, forming a thick mucus that obstructs the ducts of the different organs, with the lungs and pancreas being the most affected. This study aimed to determine the association between clinical factors and complications, as well as the survival of CF patients treated at a pediatric reference center in Mexico.

Methods: This observational, longitudinal study was conducted at the National Institute of Pediatrics in Mexico from April 2012 to April 2022. Children with cystic fibrosis were entered into the study with a nonprobabilistic sample. The variables were demographic, pulmonary and extrapulmonary complications and mortality. The association was made with chi-square and survival with Kaplan- Meir.

Results: Seventy-one patients were analyzed, including 41 infants (62%) and 12 preschoolers (16.9%). There were 40 men (56.3%). Fifty-seven cases (80.3%) had an infectious ex-acerbation, 11 cases (15.5%) had allergic bronchopulmonary aspergillosis, 9 cases (12.7%) had pulmonary hypertension, 5 cases (7%) had pneumothorax, 12 cases (16.9%) had mechanical ventilation, 70 cases (98.5%) had pancreatic insufficiency, and 10 cases (14%) had hepatobiliary disease. There was an association of extrapulmonary complications with the F508 phenotype. Overall survival was 150 months. In patients with invasive mechanical ventilation, survival was 55 months (P<0.001); in patients with noninvasive me-chanical ventilation, it was 106 months (P<0.001); in patients with pneumothorax, it was 25 months (P<0.001); and in patients with allergic bronchopulmonary aspergillosis, it was 125 months (P<0.01).

Conclusion: The most significant extrapulmonary complication in CF patients is pancreatic insufficiency. The pres-ence of pneumothorax markedly decreases survival.