Epidemiología de los adultos con diagnóstico de fibrosis quística atendidos en el área de neumología del Hospital Vicente Corral Moscoso durante el periodo enero-diciembre 2024

Abstract

Background: cystic fibrosis (CF) is an autosomal recessive genetic disease caused by mutations in the CFTR gene (ΔF508 being the most common). It is a multi-organ disease whose life expectancy has increased in recent years. In Ecuador, studies on the adult population are scarce, and there is a lack of information about this group. Objective: to describe the epidemiology of adults diagnosed with cystic fibrosis who were treated in the Pulmonology Department of the Vicente Corral Moscoso Hospital during the period January–December 2024. Methodology: observational, descriptive, retrospective, and cross-sectional study involving 13 adult patients. Data were collected through the review of medical records (MEDISYS system) and the department’s database. The data were analyzed using descriptive statistics (Epi Info™ 7.2.6 and Excel 2019). Results: the average age was 28.85 years (53,80% female), with a predominance of urban origin (76,90%) and the majority coming from Azuay (61,50%). The diagnosis was made at an average age of 16.85 years, mainly through sweat testing (84,60% positive). All patients presented with bronchiectasis (76,90% with total bilateral involvement), and 76,90% showed spirometric obstruction. The most frequent bacterial colonization was by Pseudomonas aeruginosa (53,85%) and Staphylococcus aureus (46,15%). A total of 38,46% required hospitalization due to exacerbations. Conclusions: adults with CF in this region exhibit late diagnosis, a high burden of pulmonary disease, and frequent colonization by different pathogens. These findings highlight the need to strengthen early diagnosis, access to genetic testing, and comprehensive management of this understudied population.