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.
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On 05 Jun, 2020
On 31 May, 2020
On 30 May, 2020
On 30 May, 2020
On 27 May, 2020
Received 24 May, 2020
Received 10 May, 2020
On 02 May, 2020
On 23 Apr, 2020
On 20 Apr, 2020
Invitations sent on 20 Apr, 2020
On 19 Apr, 2020
On 19 Apr, 2020
Posted 07 Oct, 2019
On 31 Mar, 2020
Received 29 Mar, 2020
On 08 Mar, 2020
Received 27 Oct, 2019
On 06 Oct, 2019
On 03 Oct, 2019
On 03 Oct, 2019
On 03 Oct, 2019
Invitations sent on 03 Oct, 2019
On 20 Sep, 2019
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.
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