ANALYSIS OF CLINICAL DATA OF ADULT PATIENTS TREATED IN CYSTIC FIBROSIS CENTER IN 2018–2020. ADULT CYSTIC FIBROSIS CENTER EXPERIENCE

Abstract

The aim of the study was to analyze the data of adult CF patients treated in the Adult Cystic Fibrosis (CF) Center of the Hospital of Lithuanian University of Health Science Kauno klinikos (Kauno klinikos) in 2018–2020 and registered in the European CF Society Patient Register (ECFSPR) and to compare the main data with the previously analyzed data of 2014–2017. Methods. A retrospective study was performed. The main demographic and clinical data of patients treated and monitored at the Adult CF Center in 2018–2020 were evaluated and compared with the analysis data of 2014–2017. Results. During the analyzed period, a total of 20 patients (12 men and 8 women) were treated at the Adult CF Center. The number of monitored patients increased (4 new patients). The average age of CF diagnosis was 10.3 ± 8.4 years. All patients had the disease confirmed genetically, and 13 had an increased concentration of chlorides in sweat. The most commonly identified mutation was ∆F508 (85%, n = 17). The average age of patients in 2018 was 27.3 ± 4.6 years; in 2019 – 27.2 ± 5.0 years; in 2020 – 27.8 ± 5.0 years. BMI in the analyzed period was 19.9 ± 2.8 kg/m2, respectively; 21.6 ± 2.6 kg/m2; 21.2 ± 2.7 kg/m2. Lung function was assessed by forced expiratory volume in the first second (FEV1), and 2018 was 54.5 ± 25.5% of predicted; in 2019 – 67.1 ± 25.7%; in 2020 – 58.4 ±29.1%. The most common chronic infection was caused by Staphylococcus aureus: 43.8% patients (n =7) in 2018; 52.6% (n = 10) in 2019; 52.6% (n = 10) in 2020; less often – Pseudomonas aeruginosa: in 2018 25.0% (n = 4); in 2019 – 10.5% (n=2); in 2020 – 10.5% (n = 2). After comparing the data from 2014 to 2020 during the period, an increase in the average age and BMI of the patients was observed, stable lung function was maintained and the frequency of detection of chronic S. aureus, P. aeruginosa and B. cepacia was decreasing. In 2020 COVID-19 infection was found in 36.8% of patients (n=7). One patient died during the analyzed period. Conclusions. In the period of 2018–2020, the number of patients at the Adult CF Center increased, and all patients had the disease confirmed genetically. The most common mutation observed was ∆F508. In the period from 2014 to 2020, the average age of patients and BMI increased and respiratory function was maintained. The most common chronic infections were caused by S. aureus and P. aeruginosa. Their frequency was decreasing. The results obtained are associated with improving care for CF patients.