Background: Cystic fibrosis-related diabetes (CFRD) is the most common comorbidity in patients with cystic fibrosis . (CF). This study aimed to determine the prevalence and risk factors of CFRD using data from the CF Registry of Türkiye (CFRT). Methods: All patients with CFRT in 2022 who underwent diabetes screening were included in the study. The clinical and demographic characteristics of patients with CFRD, pre-diabetes (impaired glucose tolerance, impaired fasting glucose, indeterminate glucose tolerance), and those with normal screening results (NSR) were compared. Results: Of the 1,702 patients registered in the data system in 2022, 916 patients from 21 centers who underwent diabetes screening were included in the study. CFRD was diagnosed in 102 patients (11.1%), there were 56 patients (6.1%) with pre-diabetes, and NSR were found in 757 patients (82.6%). The youngest patient diagnosed with diabetes was 6 years old. In patients with CFRD, the median age of patients was found to be higher (p<0.001), body mass index (BMI) was found to be lower (p 0.017); chronic Pseudomonas aeruginosa (P. aeruginosa) colonization (p<0.001), severe mutations (60.8%, p <0.001), and pancreatic insufficiency (56.1%, p<0.001) were more frequent; median FEV1% and FVC% values were significantly lower (p<0.001 and p<0.002, respectively) compared to non-diabetic patients. Conclusion: CFRD is associated with impaired pulmonary function, poor nutritional status, and increased chronic bacterial colonization of the airways. Advanced age, decreased respiratory function, severe genotype, and pancreatic insufficiency are risk factors for CFRD.

The management of respiratory problems in children on home invasive mechanical ventilation (HIMV) is a complex and challenging task. In recent years, with appropriate family education, these patients have been able to be discharged from the hospital and continue their treatment at home. The population of pediatric patients dependent on HIMV has been increasing worldwide, presenting unique and varying care needs. Management of these patients involves addressing ventilator settings, monitoring respiratory status, ensuring airway safety, and providing continuous support and education to patients and their caregivers. Despite the completion of home settings and family education, children on HIMV may encounter various respiratory problems during home follow-up. Prevention and timely management of these complications are crucial to improving patient outcomes. This article summarizes the most significant respiratory problems in children on HIMV and the management strategies for each problem are discussed, emphasizing the importance of appropriate aspiration techniques, regular monitoring, adequate training of caregivers, and a well-prepared emergency plan.