Abstract

Introduction: Even though causative factors of non-cystic fibrosis bronchiectasis (NCFB) have private pathogenesis, the general approach evaluates them under a single title NCFB.
Aim: We aimed to evaluate clinical and laboratory features of primary ciliary dyskinesia (PCD), congenital immunodeficiency syndromes (ID), and chronic aspiration (CA) diseases which are common etiologies of NCFB in children.
Methods: Retrospective data including demographics, signs and symptoms (cough, sputum, dyspnea, wheezing, hemoptysis, cyanosis, clubbing), spirometry, sputum cultures, locations of bronchiectasis in chest CT at the time of diagnosis with bronchiectasis related to PCD, ID and CA were collected.
Results: Eighty PCD, 37 ID, and 19 CA patients were included. The mean ages at the diagnosis of bronchiectasis were 10,13±3,65 years for PCD,10,78±3,04 years for ID, and 8,63±3,89 years for CA (p>0.05). Compared to other groups, those with CA had lower z-scores of FEV1 (p<0.01) and FVC (p<0.01). There was no statistically significant difference between groups by involved lung lobes. Overall, the most common symptoms were cough and sputum. Wheezing was more common in the group with PCD (p<0.01). Dyspnea was more common in groups with PCD and ID which was significantly different from the CA group (p<0.042). Overall, the most commonly isolated microorganisms were Haemophilus influenzae and Staphylococcus aureus respectively.
Conclusion: The study showed that the CA group had lower lung function and wheezing and dyspnea differed between groups. The limitation of the study was that retrospective data collection caused an unequal number of patients among groups.