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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.
Keywords
Bronchiectasis; child; primary ciliary dyskinesia; spirometry
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CURRENT STATUS: Published
View the published article at BMC Pulmonary Medicine.
No community comments so far
Editorial decision: Accept
On 11 Jun, 2020
Editor assigned
On 09 Jun, 2020
Submission checks complete
On 08 Jun, 2020
Editor invited
On 08 Jun, 2020
No comments provided
Editorial decision: Minor revision
On 05 Jun, 2020
Editor assigned
On 31 May, 2020
Submission checks complete
On 30 May, 2020
Editor invited
On 30 May, 2020
No comments provided
Editorial decision: Minor revision
On 27 May, 2020
Review #2 received
Received 24 May, 2020
Review #1 received
Received 10 May, 2020
Reviewer #2 agreed
On 02 May, 2020
Reviewer #1 agreed
On 23 Apr, 2020
Editor assigned
On 20 Apr, 2020
Reviewers invited
Invitations sent on 20 Apr, 2020
Submission checks complete
On 19 Apr, 2020
Editor invited
On 19 Apr, 2020
Version 1
Posted 07 Oct, 2019
No comments provided
Editorial decision: Major revision
On 31 Mar, 2020
Review #2 received
Received 29 Mar, 2020
Reviewer #2 agreed
On 08 Mar, 2020
Review #1 received
Received 27 Oct, 2019
Reviewer #1 agreed
On 06 Oct, 2019
Submission checks complete
On 03 Oct, 2019
Editor invited
On 03 Oct, 2019
Editor assigned
On 03 Oct, 2019
Reviewers invited
Invitations sent on 03 Oct, 2019
First submitted
On 20 Sep, 2019
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Subject Areas
Pulmonology
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See the published version of this preprint at BMC Pulmonary Medicine.
This is a preprint, a preliminary version of a manuscript that has not completed peer review at a journal. Research Square does not conduct peer review prior to posting preprints. The posting of a preprint on this server should not be interpreted as an endorsement of its validity or suitability for dissemination as established information or for guiding clinical practice.
Learn more about In Review
Research article
Badges
Prescreen
This manuscript passed our Prescreen assessment
Complete author information
Appropriate declaration statements
Potential risks to human health
Prescreen
Peer Review Timeline
CURRENT STATUS: Published
View the published article at BMC Pulmonary Medicine.
No community comments so far
Editorial decision: Accept
On 11 Jun, 2020
Editor assigned
On 09 Jun, 2020
Submission checks complete
On 08 Jun, 2020
Editor invited
On 08 Jun, 2020
No comments provided
Editorial decision: Minor revision
On 05 Jun, 2020
Editor assigned
On 31 May, 2020
Submission checks complete
On 30 May, 2020
Editor invited
On 30 May, 2020
No comments provided
Editorial decision: Minor revision
On 27 May, 2020
Review #2 received
Received 24 May, 2020
Review #1 received
Received 10 May, 2020
Reviewer #2 agreed
On 02 May, 2020
Reviewer #1 agreed
On 23 Apr, 2020
Editor assigned
On 20 Apr, 2020
Reviewers invited
Invitations sent on 20 Apr, 2020
Submission checks complete
On 19 Apr, 2020
Editor invited
On 19 Apr, 2020
Version 1
Posted 07 Oct, 2019
No comments provided
Editorial decision: Major revision
On 31 Mar, 2020
Review #2 received
Received 29 Mar, 2020
Reviewer #2 agreed
On 08 Mar, 2020
Review #1 received
Received 27 Oct, 2019
Reviewer #1 agreed
On 06 Oct, 2019
Submission checks complete
On 03 Oct, 2019
Editor invited
On 03 Oct, 2019
Editor assigned
On 03 Oct, 2019
Reviewers invited
Invitations sent on 03 Oct, 2019
First submitted
On 20 Sep, 2019
Metrics
Comments: 0
PDF Downloads: ...
HTML Views: ...
Subject Areas
Pulmonology
License:
This work is licensed under a CC BY 4.0 License. Read Full License
View the published article at BMC Pulmonary Medicine.
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.
This is a list of supplementary files associated with this preprint. Click to download.
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