Cystic Fibrosis Bronchiectasis Research Articles

Comparison of respiratory pathogen colonization and antimicrobial susceptibility in people with cystic fibrosis bronchiectasis versus non-cystic fibrosis bronchiectasis: a protocol for a systematic review

BackgroundBoth cystic fibrosis (CF) and non-cystic fibrosis bronchiectasis are characterized by permanent bronchial dilation, impaired mucociliary clearance, and development of chronic colonization and infection. Although the major airway microbiota in both CF and non-CF bronchiectasis may be similar, there are some differences in clinical and microbiologic features. There may also be differences in antibiotic susceptibility patterns between the CF and non-CF populations. Therefore, analysis and comparison of the microbiota and antibiotic susceptibility pattern in CF bronchiectasis versus non-CF bronchiectasis would help to improve the management of both conditions.MethodsTwo authors will independently search the electronic databases PubMed, EMBASE, the Cochrane Library, and LIVIVO, for studies reporting bacterial colonization of the respiratory tract in adults and children diagnosed with bronchiectasis in either CF or non-CF. We will include studies examining any respiratory tract specimen, using conventional bacterial culture or other specialized techniques such as molecular methods. We will also examine the antimicrobial susceptibility patterns in people with CF bronchiectasis versus non-CF bronchiectasis. The authors will independently assess the risk of bias in each included study using the Newcastle Ottawa Scale (NOS). We will present the data with descriptive statistics and provide pooled estimates of outcomes, wherever it is feasible to perform meta-analysis. Heterogeneity in studies will be explored by visual inspection of forest plots as well as using the Higgins and Thompson I2 method. We will contact the corresponding authors of studies where data is/are missing and try to obtain the missing data. We will undertake sensitivity analysis to explore the impact of study quality and subgroup analysis based on pre-set criteria. We will prepare a summary of findings’ table and assess the confidence in the evidence using the GRADE methodology.DiscussionTo date, there are no locally applicable evidence-based guidelines for antimicrobial treatment of non-CF bronchiectasis patients. In general, treatment is based on extrapolation of evidence in people with CF bronchiectasis. An insight into the microbiota and antimicrobial susceptibility patterns in the two conditions would facilitate appropriate rather than empiric antimicrobial therapy and hopefully reduce the burden of antimicrobial resistance created by rampant usage of antibiotics.Systematic review registrationThe protocol has been registered in PROSPERO on July 26, 2020 (PROSPERO registration number: CRD42020193859).

Validation of a Spanish version of the Leicester Cough Questionnaire in cystic fibrosis.

Cough is a main symptom in cystic fibrosis (CF). We aim to validate a Spanish version of the Leicester Cough Questionnaire (LCQ-Sp) to measure the impact of cough in CF bronchiectasis. A prospective longitudinal multicentre study was performed. Internal consistency and score changes over a 15-day period in stable state were assessed to analyse reliability. Concurrent validity was analysed by correlation with Saint George’s Respiratory Questionnaire (SGRQ) and convergent validity by assessing the association with clinical variables. Changes in scores between stable state and the first exacerbation were assessed to analyse responsiveness. 132 patients (29.73 ± 10.52 years) were enrolled in four hospitals. Internal consistency was high for the total score and good for the three domains (Cronbach’s α 0.81–0.93). The test–retest reliability showed an intraclass correlation coefficient of 0.86 for the total score. The correlation between LCQ-Sp and SGRQ scores was −0.74. The LCQ-Sp score negatively correlated with sputum volume, and the mean score decreased at the beginning of exacerbations (16.04±3.81 vs 13.91±4.29) with a large effect size. The LCQ-Sp is a reliable, repeatable and responsive instrument to assess the impact of cough in CF bronchiectasis and is responsive to change in the event of exacerbations.

Gender differences in non-cystic fibrosis bronchiectasis severity and bacterial load: the potential role of hormones.

Non cystic-fibrosis bronchiectasis (NCFB) is a complex chronic respiratory disease, characterised by excessive sputum production and abnormal permanent dilation of bronchi. Mucus accumulation leads to recurrent bacterial infections and increased bacterial load, causing vicious cycles of structural damage and decreased lung function. Respiratory physiotherapy management of NCFB includes airway clearance techniques and use of nebulised, hypertonic saline. Despite advances in treatment, a consistent relationship has been observed between gender and disease occurrence, with a higher prevalence amongst females. Furthermore, NCFB presents most aggressively amongst post-menopausal females, a group likely exposed to higher levels of progesterone (P4) over a longer period of time. The effects of gender-specific hormones on bacterial load and physiotherapy management of people living with NCFB remain unknown. The aim of this narrative review was to discuss the potential influence of gender specific hormones on NCFB disease progression and influence on physiotherapy, medical management and future research. SCOPUS and PUBMED electronic databases were used to conduct searches for relevant studies using specific inclusion and exclusion criteria. Secondary inclusion of relevant literature was obtained from primary paper references. Previous literature suggests that P4 may impair Cilia Beat Frequency (CBF) in airway epithelium. Reduction in CBF may further reduce ability to expectorate amongst individuals with NCFB, increasing bacterial load and likelihood of exacerbations, negatively impacting on disease progression. Furthermore, coadministration of Estrogen has been suggested to offer opposing effects to that of P4 only. These findings question whether hormonal levels may be monitored, controlled and optimised within management and treatment of females with NCFB to improve airway clearance, reduce exacerbations and improve quality of life. Larger scale, long-term trials are required to further explore the effects of gender specific hormones on NCFB and the viability of treatment with hormone replacement therapy.The reviews of this paper are available via the supplemental material section.

Effectiveness of hypertonic saline nebulization in airway clearance in children with non‐cystic fibrosis bronchiectasis: A randomized control trial

Failure to expectorate mucus resulting in progressive airway damage is the hallmark of bronchiectasis. Therefore effective airway clearance techniques (ACT) is the key step in its management. The aim of this study was to evaluate the efficacy of 3% hypertonic saline (HS) pre-medication in ACT in children with non cystic fibrosis (non-CF) bronchiectasis. In this randomized crossover control trial five to 15 year old children, diagnosed with non-CF bronchiectasis were randomized either to receive 200 µg of inhaled salbutamol followed by HS nebulization (test) or only 200 µg of inhaled salbutamol, before chest physiotherapy which is the conventional ACT (controls) for 8 weeks. Inhaled salbutamol was administered via a pressurized metered dosed inhaler with a valved holding chamber. After completion of first phase both groups went through one month washout period, before being crossed over to the opposite arms in the second phase. Spirometric parameters and number of exacerbations were recorded at the end of phase I, washout period and phase II. Fifty two out of 63 enrolled completed the study. Baseline characteristics of the two groups were similar. A significantly higher mean improvement was seen in predicted forced expiratory volume in 1 s in the HS arm during phase 1 (HS = 14.15 ± 5.50 vs. conventional = 5.04 ± 5.55, p = .001) and phase II (HS = 10.81 ± 5.51 vs. conventional = 3.54 ± 5.13, p = .001) compared to conventional ACT arm. HS group showed a significantly higher mean improvement in predicted forced vital capacity in phase I (HS = 13.77 ± 5.73 vs. conventional = 7.54 ± 4.90, p = .001) and phase II, (HS = 9.42 ± 7.00 vs. conventional = 4.42 ± 4.00, p = .003). Mean number of exacerbations experienced by a single child during phase I (2 months) were significantly less (p = .001) in HS arm (0.42 ± 0.64) compared to that of conventional arm (1.30 ± 1.05) butthis difference was not significant in phase II (HS = 0.65 ± 0.74 and conventional = 1.03 ± 0.77, p = .074). Incorporating HS nebulization into ACT is an effective strategy to improve dynamic lung volumes and morbidity in children with non-CF bronchiectasis.

Primary Immunodeficiency Disorders in children with Non-Cystic Fibrosis Bronchiectasis.

Introduction. Primary immunodeficiency diseases (PID) are common in patients with non-cystic fibrosis bronchiectasis (NCFB). Our objective was to determine ratio/types of PID in NCFB. Methods. Seventy NCFB patients followed up in a two-year period were enrolled. Results. Median age was 14 years (min-max: 6-30). Male/female ratio was 39/31; parental consanguinity, 38.6%. Most patients with NCFB (84.28%) had their first pulmonary infection within the first year of their lives. Patients had their first pulmonary infection at a median age of 6 months (min-max: 0.5-84), were diagnosed with bronchiectasis at about 9 years (114 months, min-max: 2-276). PID, primary ciliary dyskinesia (PCD), bronchiolitis obliterans, rheumatic/autoimmune diseases, severe congenital heart disease and tuberculosis were evaluated as the most common causes of NCFB. About 40% of patients (n=16) had bronchial hyperreactivity (BH) and asthma. Twenty-nine patients (41.4%) had a PID, and nearly all (n=28) had primary antibody deficiency, including patients with combined T and B cell deficiency. PID and non-PID groups did not differ according to gender, parental consanguinity, age at first pneumonia, age of onset of chronic pulmonary symptoms, bronchiectasis, presence of gastroesophageal reflux disease (GERD), BH and asthma (p greater-than 0.05). Admission to immunology clinic was about 3 years later in PID compared with non-PID group (p less-than 0.001). Five patients got molecular diagnosis, X-linked agammaglobulinemia (n=2), LRBA deficiency (n=1), RASGRP1 deficiency (n=1), MHC Class II deficiency (n=1). They were given monthly IVIG and HSCT was performed for three patients. Conclusions. PID accounted for about 40% of NCFB. Early diagnosis/appropriate treatment have impact on clinical course of a PID patient. Thus, follow-up in also immunology clinics should be a routine for patients who experience pneumonia in the first year of their lives and those with NCFB.

Lung Transplant Consideration: Anesthesiologist Perspective

Lung transplant has seen a significant progress since 1963 till this era. Worldwide lung transplant indications have broadened with time. Alpha 1 antitrypsin deficiency used to be the most common reason for transplant but now conditions like idiopathic pulmonary fibrosis, Cystic fibrosis, Non Cystic fibrosis bronchiectasis, lymphangioleiomyomatosis have become leading indications towards lung transplant. Relaxation of donor selection criteria management protocol preserving and optimizing lung function with development ex vivo perfusion techniques to recondition suboptimal lung has improved lung transplantation success. Post-transplant survival still poses challenge as median survival stands low around five years.

The Healthy Airway Mycobiome in Individuals of Asian Descent

The Healthy Airway Mycobiome in Individuals of Asian Descent

Hyaluronic acid for the treatment of airway diseases in children: Little evidence for few indications.

Hyaluronic acid (HA) is major physiological component of the extracellular matrix, which, in its high molecular weight form (HMW-HA) has anti-inflammatory properties. The diffusion of many different medical devices for inhalation therapy containing HA has led to an increase in their prescription, also in children. Here, we systematically review the published evidence on the efficacy and safety of HA for the treatment of upper and lower airway diseases in childhood. Relevant published studies (randomized controlled trials) for the efficacy of HA inhalation in children with upper airways diseases, asthma, cystic fibrosis (CF), and non-CF bronchiectasis were searched in Pubmed, Scopus, and Web of Knowledge databases by combining the adequate Medical Subject Headings terms and keywords, with no limit for the year of publication. We identified seven relevant publications for upper airways diseases, one for asthma, and five for CF, while we found no clinical trial including children with non-CF bronchiectasis. Meta-analysis was not conducted due to the heterogeneity of the included studies. The evidence of HA efficacy in the treatment of the upper and lower airways is still limited in children. Available data suggest that inhaled HMW-HA could be useful in the treatment of recurrent upper respiratory infections and chronic or recurrent inflammation of the middle ear and adenoids as well as of the lower airways in cystic fibrosis in association with hypertonic saline solution. Studies on larger populations and on the different formulations and nebulization methods, especially in pediatric age, are urgently needed.

Visuomotor reaction time and dynamic balance in children with cystic fibrosis and non-cystic fibrosis bronchiectasis: A case-control study.

Extrapulmonary involvement such as balance and reaction time is unclear in cystic fibrosis (CF) patients. The aim of this study was to evaluate visuomotor reaction time (VMRT) and dynamic balance in children with CF and non-CF bronchiectasis compared to healthy children. Demographic and clinical characteristics were recorded. All children were evaluated with pulmonary function test (PFT) using a spirometer, incremental shuttle walk test (ISWT) for exercise capacity, Fitlight Trainer for VMRT, and functional reach test (FRT) for dynamic balance. Fourteen children with CF (10.71 ± 2.94 years, 7 females), 17 children with non-CF bronchiectasis (12.75 ± 2.81 years, 8 females), and 21 healthy children (11.36 ± 3.28 years, 11 females) were included. Children with CF had longer total VMRT (P = .027), poorer FRT performance (P = .001), and shorter ISWT distances (P = .03) compared to the children with non-CF bronchiectasis and controls. Although total VMRT was longest in the CF group, there was no significant difference in mean VMRT among the CF, non-CF bronchiectasis, and control groups (P > .05). Dynamic balance and VMRT show greater impairment in children with CF than in children with non-CF bronchiectasis compared to healthy controls. Our findings suggest that VMRT and dynamic balance should be taken into consideration for assessments and exercise programs in pulmonary rehabilitation.

The fungal airway microbiome in cystic fibrosis and non-cystic fibrosis bronchiectasis

BackgroundThe prevalence of fungal disease in cystic fibrosis (CF) and non-CF bronchiectasis is increasing and the clinical spectrum is widening. Poor sensitivity and a lack of standard diagnostic criteria renders interpretation of culture results challenging. In order to develop effective management strategies, a more accurate and comprehensive understanding of the airways fungal microbiome is required. The study aimed to use DNA sequences from sputum to assess the load and diversity of fungi in adults with CF and non-CF bronchiectasis. MethodsNext generation sequencing of the ITS2 region was used to examine fungal community composition (n = 176) by disease and underlying clinical subgroups including allergic bronchopulmonary aspergillosis, chronic necrotizing pulmonary aspergillosis, non-tuberculous mycobacteria, and fungal bronchitis. Patients with no known active fungal disease were included as disease controls. ResultsITS2 sequencing greatly increased the detection of fungi from sputum. In patients with CF fungal diversity was lower, while burden was higher than those with non-CF bronchiectasis. The most common operational taxonomic unit (OTU) in patients with CF was Candida parapsilosis (20.4%), whereas in non-CF bronchiectasis sputum Candida albicans (21.8%) was most common. CF patients with overt fungal bronchitis were dominated by Aspergillus spp., Exophiala spp., Candida parapsilosis or Scedosporium spp. ConclusionThis study provides a framework to more accurately characterize the extended spectrum of fungal airways diseases in adult suppurative lung diseases.

P137 Further exploring phenotype and virulence differences between chronic Pseudomonas aeruginosa isolates from patients with cystic fibrosis and non-cystic fibrosis bronchiectasis (Bx)

P137 Further exploring phenotype and virulence differences between chronic Pseudomonas aeruginosa isolates from patients with cystic fibrosis and non-cystic fibrosis bronchiectasis (Bx)

Role of Stenotrophomonas maltophilia isolation in patients with non-CF bronchiectasis

Stenotrophomonas maltophilia is a bacteria whose role in patients with cystic fibrosis (CF) bronchiectasis has been previously studied; little is known about its role in non-CF bronchiectasis. Aim of our study is to investigate the risk factors for S. maltophilia acquisition and its clinical impact on bronchiectasis patients. A retrospective observational cohort study enrolling patients attending the Bronchiectasis Clinic at the Royal Infirmary of Edinburgh, Scotland, UK. A total of 167 bronchiectasis patients undergoing intravenous (IV) antibiotic therapy were selected and divided according to single or chronic S. maltophilia isolation in sputum. The risk factors and prognostic impact were studied. Single isolation was independently associated with lower baseline % predicted forced expiratory volume in 1 s [odds ratio (OR) 0.98; 95% confidence interval (CI) 0.970-1.044; P = 0.025] and with less radiological involvement (OR 0.379; 95% CI 0.175-0.819; P = 0.01). Chronic isolation was associated with the number of IV antibiotic courses in the year before and after the first isolation (OR 1.2; 95% CI 1.053-1.398; P = 0.007) and with the absence of Pseudomonas aeruginosa colonization (OR 0.207; 95% CI 0.056-0.764; P = 0.02). In the chronic isolation group, there were more exacerbations and more need of IV antibiotics in the year after the first isolation. Poor lung function is the main independent risk factor for single isolation of S. maltophilia. For chronic colonization, the main independent risk factor is the number of IV antibiotic courses and the absence of P. aeruginosa chronic colonization. Only when chronically present, S. maltophilia had a clinical impact with more exacerbations.

Non-CF bronchiectasis: Orphan disease no longer.

Bronchiectasis is a complex, chronic respiratory condition, characterized by frequent cough and exertional dyspnea due to a range of conditions that include inherited mucociliary defects, inhalational airway injury, immunodeficiency states and prior respiratory infections. For years, bronchiectasis was classified as either being caused by cystic fibrosis or non-cystic fibrosis. Non-cystic fibrosis bronchiectasis, once considered an orphan disease, is more prevalent worldwide in part due to greater availability of chest computed tomographic imaging. Identification of the cause of non-cystic fibrosis bronchiectasis with the use of chest imaging, laboratory testing, and microbiologic assessment of airway secretions can lead to initiation of specific therapies aimed at slowing disease progression. Nonpharmacologic therapies such as airway clearance techniques and pulmonary rehabilitation improve patient symptoms. Inhaled corticosteroids should not be routinely prescribed unless concomitant asthma or COPD is present. Inhaled antibiotics prescribed to individuals with >3 exacerbations per year are well tolerated, reduce airway bacteria load and may reduce the frequency of exacerbations. Likewise, chronic macrolide therapy reduces the frequency of exacerbations. Medical therapies for cystic fibrosis bronchiectasis may not be effective in treatment of non-cystic fibrosis bronchiectasis.

Deviations of body functions and structure, activity limitations, and participation restrictions of the International Classification of Functioning, Disability, and Health model in children with cystic fibrosis and non-cystic fibrosis bronchiectasis.

To the best of our knowledge, there is no study in the literature investigating the extrapulmonary outcomes of children with non-cystic fibrosis (CF) bronchiectasis and CF under the framework of the International Classification of Functioning, Disability, and Health (ICF) model. The purpose of the present study is to evaluate the children with CF and non-CF bronchiectasis using the ICF model. Children with CF, non-CF bronchiectasis, and healthy counterparts were evaluated (20 participants in each group) according to the ICF items in domain b (body functions), domain s (body structures), and domain d (activities and participation). The pulmonary functions, respiratory and peripheral muscle strength tests, and posture analysis were carried out for domain b. For domain d, however, the Glittre-activities of daily living test and Pediatric Outcome Data Collection were used. Muscle strength of shoulder abductors and hip extensors in children with CF was significantly lower than healthy children and adolescents (P < .05). The severity of lateral and posterior postural abnormalities in children with CF and non-CF bronchiectasis was higher than those of healthy children (P < .05). Among the patient groups, global function, sports/physical function, expectations, transfers/basic mobility, and pain/comfort were the most affected participation dimensions (P < .05). This study highlights the need for comprehensive up-to-date evaluation methods according to the ICF model for understanding rehabilitation requirements in CF and non-CF bronchiectasis in different age groups.

Airway mucus hyperconcentration in non–cystic fibrosis bronchiectasis

Airway mucus hyperconcentration in non–cystic fibrosis bronchiectasis

The Role of Vitamin D3 Therapy in Pediatric Bronchiectasis Severity (CF versus Non-CF Patients)

Objective: To determine and compare the effect of vitamin D3 supplementation on modifying the disease severity in cystic fibrosis (CF) and non-CF bronchiectasis pediatric patients. Methods: A randomized clinical trial evaluating the role of oral vitamin D3 supplementation for six months, was performed in forty patients with CF and non-CF bronchiectasis under the age of 18 years with vitamin D deficiency or insufficiency. The primary outcome was to reach the sufficient Vitamin D level, the secondary outcome was to reevaluate bronchiectasis severity by following up the frequency, severity of pulmonary exacerbations and lung function after vitamin D3 supplementation. Results: Forty patients completed the trial. The percentage of improvement of vitamin D level after vitamin D3 supplementation for six months was significantly higher in CF (88.3%) than non-CF bronchiectasis patients (59.82%) (P = 0.03). Additionally, moderate to severe pulmonary exacerbations significantly decreased by more than 60%, 45% (P = 0.001, 0.005) and frequent exacerbations decreased by 15%, 10% (P = 0.327, 0.490), while the forced expiratory volume in 1 (FEV1) significantly increased by 17% and 15% in non CF bronchiectasis and CF patients respectively (p 0.001). Conclusions: Vitamin D3 therapy was effective in decreasing the frequency and severity of pulmonary exacerbations and preserving lung function. Thereby, improving the disease severity even more in non-CF bronchiectasis than CF patients.

Nontuberculous mycobacterium as a hidden cause of noncystic fibrosis bronchiectasis

Background The incidence of lung colonization and infection owing to nontuberculous mycobacteria (NTM) in patients with cystic fibrosis (CF) and non-CF bronchiectasis is increasing worldwide, which may play a role in pathogensis of bronchiectasis. Aim The aim of this study is to search about NTM as a hidden cause of non-CF bronchiectasis. Patients and methods This is an observational cohort study done at Chest Department of Menoufia University Hospital on 40 patients with non-CF bronchiectasis more than 18 years of age between June 2017 and June 2018. All patients underwent spirometry, chest radiograph, high-resolution computed tomography, and sputum examination for tuberculous and NTM. The sputum samples were assessed by PCR, and positive cases did another PCR after 2 months. Results A total of 40 (21 females and 19 males) patients were included in the study, with mean age of 55.2±6.5 years, mean BMI of 21.4±2.1, and mean height of 163.7±7.3 cm. There were 7.5% of non-CF bronchiectasis cases with NTM (n=3). Mycobacterium avium complex (MAC) was the most common mycobacteria (66.6%) of positive patients. NTM isolation was significantly higher in elderly, tall, low BMI, and female patients (66%). High-resolution computed tomography of patients with NTM especially MAC has shown mucous plugging, peripheral nodules, and ectatic changes in bronchi, and also sputum purulence was less in patients positive for NTM. Conclusion NTM, especially MAC, may be a hidden cause in non-CF bronchiectasis and must be treated properly.

The Fungal Airway Microbiome in Cystic Fibrosis and Bronchiectasis

Background: The prevalence of fungal disease in cystic fibrosis (CF) and non-CF bronchiectasis is increasing and the clinical spectrum is widening. Poor sensitivity and a lack of standard diagnostic criteria renders interpretation of culture results challenging. In order to develop effective management strategies, a more accurate and comprehensive understanding of the airways fungal microbiome is required. The study aimed to use DNA sequences from sputum to assess the load and diversity of fungi in adults with CF and bronchiectasis. Methods: Next generation sequencing of the ITS2 region was used to examine fungal community composition (n = 176) by disease and underlying clinical subgroups including allergic bronchopulmonary aspergillosis, chronic necrotizing pulmonary aspergillosis, non-tuberculous mycobacteria, and fungal bronchitis. Patients with no known active fungal disease were included as disease controls. Findings: ITS2 sequencing greatly increased the detection of fungi from sputum. In patients with CF fungal diversity was lower, while burden was higher than those with bronchiectasis. The most common operational taxonomic unit (OTU) in patients with CF was Candida parapsilosis (20·4%), whereas in bronchiectasis sputum Candida albicans (21·8%) was most common. CF patients with overt fungal bronchitis were dominated by Aspergillus spp., Exophiala spp., Candida parapsilosis or Scedosporium spp. This study provides a framework to more accurately characterize the extended spectrum of fungal airways diseases in adult suppurative lung diseases. Funding Statement: The project was supported by the Asmarley Trust, the Wellcome Trust and the NIHR Respiratory Disease Biomedical Research Unit at the Royal Brompton and Harefield NHS Foundation Trust, Imperial College London. IF was supported by an NIHR PhD studentship. Declaration of Interests: The authors have no conflicts of interests to disclose. Ethics Approval Statement: The study was approved by the Royal Brompton and Harefield Hospital Biomedical Research Unit Ethics Committee (Advanced Lung Disease Biobank Study Number: 10/H0504/9).

Infection prevention and chronic disease management in cystic fibrosis and noncystic fibrosis bronchiectasis.

Bronchiectasis is a chronic lung disease (CLD) characterized by irreversible bronchial dilatation noted on computed tomography associated with chronic cough, ongoing viscid sputum production, and recurrent pulmonary infections. Patients with bronchiectasis can be classified into two groups: those with cystic fibrosis and those without cystic fibrosis. Individuals with either cystic fibrosis related bronchiectasis (CFRB) or noncystic fibrosis related bronchiectasis (NCFRB) experience continuous airway inflammation and suffer airway architectural changes that foster the acquisition of a unique polymicrobial community. The presence of microorganisms increases airway inflammation, triggers pulmonary exacerbations (PEx), reduces quality of life (QOL), and, in some cases, is an independent risk factor for increased mortality. As there is no cure for either condition, prevention and control of infection is paramount. Such an undertaking incorporates patient/family and healthcare team education, immunoprophylaxis, microorganism source control, antimicrobial chemoprophylaxis, organism eradication, daily pulmonary disease management, and, in some cases, thoracic surgery. This review is a summary of recommendations aimed to thwart patient acquisition of pathologic organisms, and those therapies known to mitigate the effects of chronic airway infection. A thorough discussion of airway clearance techniques and treatment of or screening for nontuberculous mycobacteria (NTM) is beyond the scope of this discussion.

Systematic Review on Adherence, Barriers to Treatment and Impact of Airway Clearance in Bronchiectasis

Background and objective: Chest physiotherapy (CPT) is an integral standard of treatment for patients with bronchiectasis. This study aims to analyse patients’ adherence to CPT, barriers to adherence and the impact on respiratory outcomes. Methods: A systematic literature review was conducted through electronic searches of Pubmed, Embase and Cochrane databases from 1st January 2010 to 20th March 2020 for adherence and or non-adherence rates to CPT of patients with cystic fibrosis (CF) and non-CF bronchiectasis (NCFB). Results: A total of thirteen publications with a study duration ranging from 1 to 16 months were qualified for inclusion. The paediatric population had a higher mean adherence rate than the adult population (range: 51% to 61% vs. 41% to 51%). The commonest CPT modalities were positive expiratory pressure (PEP) and flutter devices. Patients with high adherence of CPT had a significantly lower rate of hospitalisation than patients with low adherence rate (0.5 episodes/year vs. 1.4 episodes/year). A low adherence to CPT was associated with more pulmonary exacerbations. Patients with higher burden of treatment, perceived necessity of CPT and higher socio-economic status were more adherent to CPT. However, the effect of adherence to CPT on lung function and the quality of life (QoL) was inconclusive. Conclusions: The current literature suggests that adherence rate to CPT is poor amongst patients with NCFB and CF. There is a greater need for better understanding of the clinical consequences and economic impact of CPT on patients with NCFB.