Background: Primary ciliary dyskinesia (PCD) is associated with ventilation defects and heterogeneous impairment of pulmonary function. Spirometry may underestimate PCD severity and complexity. This study aimed to evaluate spirometry, lung clearance index (LCI), and impulse oscillometry (IOS) in children with PCD and healthy controls, and compare them in terms of early detection of lung disease. Methods: In this cross-sectional, prospective study, participants included children aged 6-18 years with PCD and healthy age-matched controls. Lung function tests using LCI, IOS, and spirometry were conducted on the same day for all participants. Results: Thirty-two children with PCD (median age 13.19 years) and 44 age-matched healthy controls (median age 12.32 years) were studied. PCD was associated with lower FEV1, FVC, FEV1/FVC, R5, R10, R15, R20, X5, Fres, and LCI 2.5% mean values (p<0.05). Abnormal LCI 2.5% was found in 46.5% of patients with predicted FEV1 > 80%. Significant inverse correlations were observed between LCI 2.5%, FEV1, FVC, and Fres in PCD patients (p<0.001, r:-0.635; p=0.002, r:-0.517; p=0.006, r:-0.479; respectively. Conclusion: This is the first study to compare LCI, IOS, and spirometry in children with PCD. The study has shown that there are significant differences in spirometry, LCI, and IOS values between children with PCD and healthy controls. LCI can detect airway anomalies earlier than spirometry in PCD patients. IOS and LCI are valuable respiratory function tests that can be used in PCD follow-up.

Background: Previously, Cystic Fibrosis (CF) patients faced a limited life expectancy, but significant medical advances now highlight the need for successful transition programs from pediatric to adult care. Methods: The aim of this project was to implement the CF R.I.S.E. program, a structured transition program, in a CF center with limited resources at Marmara University. The program was adapted and translated into Turkish with the permission of the Cystic Fibrosis Foundation. A multidisciplinary team collaborated in the translation and adaptation process and educational materials were developed for patients and families. Results: Successful implementation of the CF RISE program was achieved within six months. A pilot study with randomly selected patients revealed positive feedback indicating the effectiveness and understandability of the program. The program facilitated strong collaboration between pediatric pulmonologists, CF nurses, dietitians and patient representatives. However, challenges were encountered due to the lack of a designated social worker, which affected patients’ access to expert guidance on social security and disability rights. Conclusions: The CF S.O.B.E. program was successfully adapted and implemented at the Marmara University CF Center in Turkey. The program is expected to have a positive impact on patients’ knowledge and self-care skills over a period of 1.5 years. It is aimed to make the program a routine practice in the center and to expand the collaboration with adult clinics. Further studies are needed to assess its long-term impact and applicability in different health settings. The ultimate goal is to disseminate the program’s resources and promote structured transition practices nationwide.

Background Cystic fibrosis (CF) is a chronic disease causing recurrent respiratory tract infections. Viral respiratory tract infections are more severe in CF. The first case of COVID-19 was seen in Turkey on March 11, 2020 and nationwide school closure and lockdown were implemented. School closure and home confinement might have adverse effects on children’s physical and mental health. In this study, we aimed to compare the effect of COVID-19 pandemic on psychological reactions of CF patients and healthy controls. Methods This is a controlled cross-sectional study including 7-18 year-old children with CF. The survey included questions regarding family environment and peer relations, self care and psychological reactions to COVID-19 pandemic. The questionnaire was applied to children via telephone call under parental supervision. Results We evaluated 132 CF patients and 135 their healthy peers. Mean age was 11.5±2.9 years in CF group and 11.8±3.2 years in control group (p=0.98). There were 55 girls (41.7%) in CF group and 81 girls (60%) in control group (p=0.027). The socioeconomic status of families was similar. CF patients was feeling less anxious about the COVID-19 pandemic compared to controls (p=0.01). CF patients were found to be less anxious for family members having the risk of COVID-19, less upset for the school closure, less anxious about the COVID-19 pandemic (p<0.001, 0.02, 0.01 respectively). Conclusion CF patients seem to show more resilience in coping with the pandemic. Appropriate psychological support should be provided to them and resilience strategies in coping with the pandemic should be nurtured.