Comparison Of Clinical Characteristics Between Two Non-Cystic Fibrosis Bronchiectasis Children Cohorts Followed Up From A Single Tertiary Center At Different Time Intervals

Abstract Background The diagnosis of non-cystic fibrosis (CF) bronchiectasis is increasing in both developed and nondeveloped countries in recent years. Although the main features are similar, etiologies seem to change. Our aim was to evaluate the clinical and laboratory characteristics of non-CF bronchiectasis patients and to compare these data with our previous cohort in 2001. Methods 104 children with non-CF bronchiectasis being followed-up between 2002 and 2019 were enrolled. Age of diagnosis, underlying etiology and distribution of microorganisms in sputum culture were recorded. Clinical outcomes were evaluated in terms of lung function tests and annual pulmonary exacerbation rates at presentation and within the last 12 months of the follow-up. Results Mean FEV1 and FVC % of predicted at presentation were improved compared with previous study (76.6±17.1 vs. 63.3±22.1 and 76.6±15.1 vs. 67.3±23.1, respectively; p<0.001). There was a significant decrase in pulmonary exacerbation rate from 6.05±2.88 at presentation to 3.23±2.08 during follow-up (p<0.0001). In 80.8% of patients, an underlying etiology was identified. There was an increase in primary ciliary dyskinesia (PCD) (32.7% vs. 6.3%;p:0.001), decrease in idiopathic cases (19.2% vs. 37.8%;p: 0.03) with no change in postinfectious and immunodeficiencies. Sputum cultures were positive in 77.9% of patients which was 46.9% in the previous study (p=0.001). Conclusion Baseline pulmonary function tests were better and distribution of underlying etiology had changed with a remarkable increase in diagnosis of PCD. Recent advances in diagnosis also makes it easier to evaluate the patients in a better way, still awaiting for international consensus and guidelines on non-CF bronchiectasis in children.