ANALYSIS OF DEMOGRAPHIC AND CLINICAL DATA OF PATIENTS TREATED AT THE ADULT CYSTIC FIBROSIS CENTRE, DEPARTMENT OF PULMONOLOGY, HOSPITAL OF LITHUANIAN UNIVERSITY OF HEALTH SCIENCES KAUNO KLINIKOS, 2012–2023

Abstract

Aim of the study. To analyse and summarise the demographic and clinical data of patients with cystic fibrosis (CF) who were treated at the Adult Cystic Fibrosis Centre of the Hospital of Lithuanian University of Health Sciences Kauno klinikos and registered in the European Cystic Fibrosis Society Patient Registry (ECFSPR) between 2012 and 2023, and to compare these data with ECFSPR data from other countries. Methods. A retrospective study was conducted. Demographic and clinical data of patients treated at the Adult Cystic Fibrosis Centre and registered in the ECFSPR from 2012 to 2023 were compared with data from other countries. Results. During the study period, 36 patients (20 men and 16 women) were treated at the Adult Cystic Fibrosis Centre. Eight patients died, and 23 new patients were registered. The average age at CF diagnosis was 8.0 ± 3.5 years, and all diagnoses were confirmed by genetic testing. The most common CFTR gene mutation was ΔF508 (83.3%, n = 30). The average patient age (years) from 2012 to 2023 was: 23.5 ± 3.3, 23.9 ± 3.7, 24.6 ± 3.7, 25.4 ± 4.1, 26.9 ± 4.2, 27.8 ± 4.0, 27.3 ± 4.6, 27.2 ± 5.0, 27.8 ± 5.0, 26.7 ± 7.3, 27.0 ± 6.3, 26.9 ± 6.7. The average BMI (kg/m²): 18.5 ± 3.3, 20.0 ± 2.4, 19.6 ± 2.2, 19.7 ± 2.8, 20.1 ± 2.6, 19.6 ± 2.5, 19.9 ± 2.8, 21.6 ± 2.6, 21.2 ± 2.7, 20.4 ± 3.4, 20.5 ± 2.8. The average FEV1 (%): 60.9 ± 28.4, 61.8 ± 27.7, 59.7 ± 27.5, 63.9 ± 29.4, 63.8 ± 23.9, 60.5 ± 26.7, 54.5 ± 25.5, 67.1 ± 25.7, 58.4 ± 27.8, 59.5 ± 27.0, 59.8 ± 29.7, 53.5 ± 28.0. The most common chronic infections were caused by Staphylococcus aureus (%), respectively 61.5, 69.2, 75.0, 57.1, 50.0, 64.3, 43.8, 58.8, 47.6, 46.2, 46.2, 50.0. All identified cases of S. aureus were methicillin-sensitive. The second most common cause of chronic infections was Pseudomonas aeruginosa (%), respectively 23.1, 7.7, 8.3, 14.3, 14.3, 14.3, 25.0, 11.8, 9.5, 15.4, 30.8, 28.6. The most frequently prescribed medications were dornase alfa and pancreatic enzymes. The most common CF-related complication was bronchiectasis. Conclusions. From 2012 to 2023, the number of patients monitored and treated at the Adult Cystic Fibrosis Centre of the Hospital of Lithuanian University of Health Sciences Kauno klinikos increased. CF was genetically confirmed in all patients, with ΔF508 being the most common mutation. An increase in life expectancy was observed, while respiratory function and BMI remained stable. The most frequent chronic infections were caused by S. aureus and P. aeruginosa, and the most prescribed medications were dornase alfa and pancreatic enzymes. These outcomes are attributed to improved diagnostic capabilities, better patient care, easier access to treatment, and multidisciplinary management.