Akademik Veri Yönetim Sistemi
European Journal of Pediatrics, cilt.185, sa.4, 2026 (SCI-Expanded, Scopus)
Childhood interstitial lung diseases (chILD) include a diverse range of rare disorders, complex clinical progressions, and high rates of morbidity and mortality. This study aims to assess the clinical and psychosocial impacts of chILD in children registered in the National chILD-Turkiye database. Data from 17 centers were analyzed as of January 2024. Patients aged 1 month to 18 years were included in the study. Through face-to-face interviews, patients and their families completed the Pediatric Quality of Life Inventory (PedsQL™ 4.0 Generic Core Scales) and PedsQL™ 2.0 Family Impact Module. Higher scores suggest better performance and higher health-related quality of life (HRQoL). Patient clinic data, demographic information, pulmonary function tests, Fan scores, and household income were recorded. Patients were categorized into two groups according to their use of O2 therapy and diffuse progressive lung disease (DPLD) groups A and B. The study included 175 patients (90 girls), with a median age of 7.7 (1.0–17.8) years. A strong positive correlation was found between the FEV1% value and both the Physical Health Summary Score and the Total Score (p = 0.007, r = 0.608; p = 0.008, r = 0.607). The Fan score negatively correlated with PedsQL™ scores (p < 0.001). Patients who use O2 have an earlier diagnosis of age of chILD, lower FEV1%, higher Fan scores, and lower Child Self-Reports and Parent Reports scores (p < 0.05). The group with DPLD-A disorders has an earlier diagnosis age, lower weight and BMI z-scores, higher Fan score, and higher Family Impact Module score (p < 0.05). Conclusion: Lung function and Fan score strongly correlate with HRQoL, emphasizing the impact of disease severity on physical and psychosocial well-being. These findings highlight the need for multidisciplinary care and psychosocial support to enhance the QoL in children with chILD and their families. (Table presented.)