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Additional file 4: Table S1. Univariate Cox regression and multivariate Cox regression analysis of the factors associated with recurrent hemoptysis of patients with idiopathic bronchiectasis after BAE. Table S2. Univariate Cox regression and multivariate Cox regression analysis of the factors associated with recurrent severe hemoptysis of patients with idiopathic bronchiectasis after BAE.

Additional file 3: Figure S3. Specific origination, the recurrence rate, and the severe recurrence rate of abnormal AbBAs on CTA. (A) The proportion of certain abnormal AbBAs on CTA to all abnormal AbBAs on CTA. (B) Ratio of recurrent hemoptysis group to all patients per abnormal AbBAs on CTA. (C) Ratio of recurrent severe hemoptysis group to all patients per abnormal AbBAs on CTA.

Additional file 1: Figure S1. The cumulative rates of patients with recurrent hemoptysis-free or recurrent severe hemoptysis-free with Pseudomonas aeruginosa, abnormal AbBAs on CTA, extensive bronchiectasis, and high 24-h sputum volume.

Additional file 2: Figure S2. Distribution characteristics of abnormal arteries in 588 patients. BAs, bronchial arteries; NBSAs, non-bronchial system arteries.

Additional file 1. Table S1. Excluded articles after full-text review. Table S2. Study characteristics of included studies. Table S3. Clinical characteristics and laboratory results of patients who have a clinical diagnosis of PCD in excluded studies after full-text review.

Wei-Jie Guan,1,2 Jing-Jing Yuan,1 Hui-Min Li,1 Yong-Hua Gao,3 Chun-Lan Chen,1 Yan Huang,1 Rong-Chang Chen,1 Nan-Shan Zhong1,2 1State Key Laboratory of Respiratory Disease, National Clinical Research Center for Respiratory Disease, Guangzhou Institute of Respiratory Health, The First Affiliated Hospital of Guangzhou Medical University, Guangzhou, People’s Republic of China; 2Sino-French Hoffmann Institute, Guangzhou Medical University, Guangzhou, People’s Republic of China; 3Department of Respiratory and Critical Care Medicine, First Affiliated Hospital of Zhengzhou University, Zhengzhou, People’s Republic of China Background: Bronchiectasis is a debilitating disease with chronic airway infection. Proteobacteria, the dominant phylum, can be detected with high-throughput sequencing.Objective: To stratify Proteobacteria compositions according to culture findings in bronchiectasis.Patients and methods: We sampled sputum, split for culture and 16srRNA sequencing, from 106 patients with stable bronchiectasis and 17 healthy subjects. Paired sputa from 22 bronchiectasis patients were collected during exacerbations and convalescence.Results: Forty-five, 41, and 20 patients with clinically stable bronchiectasis had isolated Pseudomonas aeruginosa (PA), other potentially pathogenic microorganisms, and commensals at the initial visit, respectively. The PA group (but not other groups) demonstrated significantly greater relative abundance of Proteobacteria, and lower Shannon–Wiener Diversity Index, Simpson Diversity Index, and richness compared with healthy subjects. Pseudomonas was the dominant genus that discriminated bronchiectasis patients (particularly in the PA group) from healthy subjects. Compared with baseline levels, Proteobacteria community compositions in the PA group, but not in other groups, were more resilient during exacerbations and convalescence.Conclusion: Proteobacteria community compositions could be partially reflected by conventional sputum bacterial culture. Significantly altered Proteobacteria community compositions – particularly, the increased relative abundance of Pseudomonas and diminished community diversity – represent critical targets for novel interventions to restore normal airway microenvironment in patients with bronchiectasis. Keywords: bronchiectasis, Proteobacteria, Pseudomonas aeruginosa, culture, exacerbation

Yong-hua Gao,1,* Wei-jie Guan,2,* Ya-nan Zhu,3 Rong-chang Chen,2 Guo-jun Zhang1 1Department of Respiratory and Critical Care Medicine, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, Henan, 2State Key Laboratory of Respiratory Disease, National Clinical Research Center for Respiratory Disease, Guangzhou Institute of Respiratory Diseases, The First Affiliated Hospital of Guangzhou Medical University, Guangzhou, Guangdong, 3Department of Emergency Medicine, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, Henan, People’s Republic of China *These authors contributed equally to this work Background and aims: Pseudomonas aeruginosa (PA) is the most common pathogen in bronchiectasis and frequently develops resistance to multiple classes of antibiotics, but little is known about the clinical impacts of PA-resistant (PA-R) isolates on bronchiectasis. We, therefore, investigated the prevalence, risk factors and prognostic implications of PA-R isolates in hospitalized bronchiectasis patients.Patients and methods: Between June 2011 and July 2016, data from adult bronchiectasis patients isolated with PA at the First Affiliated Hospital of Zhengzhou University were retrospectively analyzed. PA was classified as PA-R in case antibiogram demonstrated resistance on at least one occasion.Results: Seven hundred forty-seven bronchiectasis patients were assessed. Of these, 147 (19.7%) had PA isolate in the sputum or bronchoscopic culture. PA-R and PA-sensitive accounted for 88 (59.9%) and 59 (31.1%) patients, respectively. In multivariate model, factors associated with PA-R isolate in bronchiectasis included prior exposure to antibiotics (odds ratio [OR] =6.18), three or more exacerbations in the previous year (OR =2.81), higher modified Medical Research Council dyspnea scores (OR =1.93) and greater radiologic severity (OR =1.15). During follow-up (median: 26 months; interquartile range: 6–59 months), 36 patients died, of whom 24 (66.7%) had PA-R isolate at baseline. However, PA-R isolate was not associated with greater all-cause mortality in bronchiectasis.Conclusion: PA-R infection is common among bronchiectasis patients, mainly determined by prior exposure to antibiotics, frequent exacerbations, more pronounced dyspnea and more severe radiologic involvement. However, PA-R isolate is not an independent risk factor for all-cause mortality in bronchiectasis. Keywords: bronchiectasis, antibiotic resistance, Pseudomonas aeruginosa, risk factors, mortality

Bronchial dilation testing is an important tool to assess airway reversibility in adults with bronchiectasis. This study aims to investigate the association of bronchodilator response (BDR) and clinical parameters in bronchiectasis, and the utility of BDR to indicate lung function decline and risks of bronchiectasis exacerbations (BEs).We recruited 129 patients with clinically stable bronchiectasis. Baseline measurements included assessment of sputum inflammation and matrix metalloproteinase-8 and -9, sputum bacterial culture, spirometry, bronchial dilation test (for baseline FEV1 less than 80% predicted only) and chest high-resolution computed tomography (HRCT). Bronchiectasis patients were followed-up for 1 year to determine the incidence of BEs and lung function trajectories. Significant BDR was defined as FEV1 improvement from pre-dose value by at least 200 mL and 12%. Clinical trial registry No.: NCT01761214; URL: www.clinicaltrials.gov.BDR was negatively correlated with baseline FEV1 percentage predicted, but not blood or sputum eosinophil count. Significant BDR was not associated with greater proportion of never-smokers, poorer past history, greater HRCT scores, poorer diffusing capacity or increased sputum matrix metalloproteinases (all P0.05). There was a trend towards higher bronchiectasis severity index (BSI) and greater proportion of patients with Pseudomonas aeruginosa isolation or infection. Significant BDR at baseline was linked to poorer spirometry, but not more rapid lung function decline, throughout follow-up. Patients with significant BDR demonstrated non-significantly lower risks of experiencing the first BEs than those without (P=0.09 for log-rank test).Significant BDR is associated with poorer lung function compared with non-significant BDR. Whether BDR predicts future risks of BEs needs to be tested in a larger cohort.

Extended quark distribution functions are presented obtained by fitting a large amount of experimental data of the l-A DIS process on the basis of an improved nuclear density model. The experimental data of l-A DIS processes with A ≥ 3 in the region 0.0010 ≤ x ≥ 0.9500 are quite satisfactorily described by using the extended formulae. Our knowledge of the influence of nuclear matter on the quark distributions is deepened.