Bronchopulmonary colonization patterns in Spanish people with cystic fibrosis: Results of a national multicentre study

Abstract

Background: We investigate bronchopulmonary colonization patterns in Spanish people with CF (pwCF), gathering clinical, demographic, and microbiological data to supplement nine years of registry information, comparing 2021 findings with a similar multicenter study conducted in 2013.

Methods: Sixteen CF units from 14 hospitals across Spain participated, each randomly recruiting around 20 patients. Patients provided sputum samples for culture. The clinical, demographical, microbiological, and treatment data from the previous year were recorded.

Results: Overall, 326 patients (48.5% females) were recruited: 185 adult and 141 pediatrics, with a median age [q3-q1] of 30 [38-24] and 12 [16-6] years, respectively. p.Phe508del mutation was present in 30.6% and 46.4% of patients with homozygosis or heterozygosis, respectively. Median FEV₁ (%) was significantly lower in adults (62%, range 75-43%) compared with pediatrics (90%, range 104-81%) (p<0.001). Pancreatic insufficiency was observed in 77.3%, carbohydrate metabolism alteration in 27.3%, and CF-related diabetes in 19.6% of patients. Lower prevalence of Staphylococcus aureus and Pseudomonas aeruginosa colonization were noted compared to 2013, along with a significantly lower correlation in lung function among pwCF colonized by P. aeruginosa (p<0.001). Half of pwCF (51%) exhibited a single pathogen in culture, two in 30%, and three or more in 2.4%. Co-colonization of P. aeruginosa and S. aureus (36.1%) was the most prevalent combination. High resistance rates were observed in P. aeruginosa and methicillin resistant S. aureus isolates.

Conclusions: We provide a valuable and representative current insight into the observed evolution in the clinical, demographic, and microbiological aspects in recent years among pwCF in Spain.

Keywords: Bronchopulmonary colonization; Colonización broncopulmonar; Cystic fibrosis; Fibrosis quística; P. aeruginosa; Patient registry; Pseudomonas aeruginosa; Registro de pacientes; S. aureus; Staphylococcus aureus.