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Cystic and Non-Cystic, Fibrosis Bronchiectasis: Drifting from Molecular to Clinical Practice

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A special issue of Journal of Clinical Medicine (ISSN 2077-0383). This special issue belongs to the section "Pulmonology".

Deadline for manuscript submissions: closed (30 September 2020) | Viewed by 27099

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Special Issue Editors

Dr. Adelina Amorim

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Guest Editor

1. Pulmonology Department, Centro Hospitalar e Universitário de São João, Porto, Portugal
2. Faculty of Medicine, University of Porto, Porto, Portugal
Interests: bronchiectasis; cystic fibrosis; chronic lung infections; non-tuberculosis mycobacteria

Dr. Margarida Redondo

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Guest Editor

Pulmonology Department, Centro Hospitalar e Universitário de São João, Porto, Portugal
Interests: bronchiectasis; cystic fibrosis; chronic lung infections; non-invasive ventilation; neuromuscular diseases; sleep medicine

Dr. David Araújo

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Guest Editor

Pulmonology Department, Centro Hospitalar e Universitário de São João, Porto, Portugal
Interests: bronchiectasis; chronic lung infections; non-tuberculosis mycobacteria; oncology; lung cancer

Prof. Dr. Natália Cruz-Martins

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Guest Editor

1. Faculty of Medicine, University of Porto, 4099-002 Porto, Portugal
2. Institute for Research and Inovation in Health (i3S), University of Porto, 4099-002 Porto, Portugal
Interests: evidence-based medicine; phytochemistry; phytopharmacology; drug discovery; natural products biochemistry; bioactive molecules; functional foods; nutraceuticals; fungal and bacterial infections; resistance to antimicrobials
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Special Issue Information

Dear Colleagues,

In recent years, there has been increasing attention to non-cystic fibrosis (CF) bronchiectasis. For CF, the large number of studies performed in this area has led to a significant improvement in the overall life expectancy and quality of life of these patients, by using increasingly specific and effective therapeutic strategies. These advances, by allowing young patients to live longer and with better quality of life, present challenges to adulthood intervention.

On the other hand, non-CF bronchiectasis has been conceived of as one of the most neglected diseases in respiratory medicine. In fact, few studies have been done on this subject, so there is an urgent need to deepen our knowledge and address this complex chronic condition. In addition, the incidence of non-CF bronchiectasis is more frequent than what clinicians expected. Indeed, according to epidemiological data, a marked raise in bronchiectasis’ all-cause morbimortality has been stated, mainly due to multiple and recurrent exacerbations, triggered by complex infectious statuses and other complications. Thus, considering this scenario, there is an urgent need to carry out studies that allow us to more clearly understand the following: (1) the etiology underlying each case, since there is still a large percentage of patients considered idiopathic, and (2) the mechanisms associated with chronic infection and repetitive exacerbations, in order to find more effective strategies to combat them.

In this sense, all submissions addressing all of the above highlighted aspects on CF and non-CF bronchiectasis are welcome to this Special Issue. Thus, original articles, comprehensive and critical reviews, case reports, and even letters are of utmost interest, given the expansive scope of this area.

Dr. Adelina Amorim
Dr. Margarida Redondo
Dr. David Araújo
Dr. Natália Martins
Guest Editors

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Keywords

Cystic fibrosis
Bronchiectasis
Chronic infections
Exacerbations
Quality of life
Therapeutic strategies
Epidemiology
Pulmonary rehabilitation
Inhaled therapy

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Published Papers (6 papers)

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Research

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15 pages, 3682 KiB

Open AccessArticle

Systemic Inflammation during and after Bronchiectasis Exacerbations: Impact of Pseudomonas aeruginosa

by Rosario Menéndez, Raúl Méndez, Isabel Amara-Elori, Soledad Reyes, Beatriz Montull, Laura Feced, Ricardo Alonso, Rosanel Amaro, Victoria Alcaraz, Laia Fernandez-Barat and Antoni Torres

J. Clin. Med. 2020, 9(8), 2631; https://doi.org/10.3390/jcm9082631 - 13 Aug 2020

Cited by 22 | Viewed by 3543

Abstract

Bronchiectasis is a chronic structural disease associated with exacerbations that provoke systemic inflammation. We aimed to evaluate the systemic acute proinflammatory cytokine and its biomarker profiles during and after exacerbations and its relationship with the severity of episode, microbiological findings, and the bronchiectasis severity index. This prospective observational study compared exacerbation and stable groups. Cytokine (interleukins (IL)-17a, IL-1β, IL-6, IL 8; tumor necrosis factor-alpha (α)) and high-sensitivity C-reactive protein (hsCRP) levels were determined by multiplex analysis on days 1, 5, 30, and 60 in the exacerbation group and on day 1 in the stable group. We recruited 165 patients with exacerbations, of which 93 were severe (hospitalized). Proinflammatory systemic IL-17a, IL-1β, IL-8, and tumor necrosis factor-α levels increased similarly on days 1 and 5 in severe and non-severe episodes, but on day 30, IL-17a, IL-8, and IL-6 levels were only increased for severe exacerbations. The highest IL-17a level occurred in patients with chronic plus the acute isolation of Pseudomonas aeruginosa. At 30 days, severe exacerbations were independently associated with higher levels of IL-17 (Odds ratio (OR) 4.58), IL-6 (OR 4.89), IL-8 (OR 3.08), and hsCRP (OR 6.7), adjusted for age, the bronchiectasis severity index, and treatment duration. Exacerbations in patients with chronic P. aeruginosa infection were associated with an increase in IL-17 and IL-6 at 30 days (ORs 7.47 and 3.44, respectively). Severe exacerbations elicit a higher systemic proinflammatory response that is sustained to day 30. Patients with chronic P. aeruginosa infection had impaired IL-17a reduction. IL-17a could be a useful target for measuring systemic inflammation. Full article

(This article belongs to the Special Issue Cystic and Non-Cystic, Fibrosis Bronchiectasis: Drifting from Molecular to Clinical Practice)

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11 pages, 822 KiB

Open AccessArticle

Inhaled Dry Powder Antibiotics in Patients with Non-Cystic Fibrosis Bronchiectasis: Efficacy and Safety in a Real-Life Study

by Miguel Ángel Martínez-García, Grace Oscullo, Esther Barreiro, Selene Cuenca, Angela Cervera, Alicia Padilla-Galo, David de la Rosa, Annie Navarro, Rosa Giron, Francisco Carbonero, Maria Castro Otero and Francisco Casas

J. Clin. Med. 2020, 9(7), 2317; https://doi.org/10.3390/jcm9072317 - 21 Jul 2020

Cited by 6 | Viewed by 4017

Abstract

Background: Nebulised antibiotics are habitually used in patients with bronchiectasis, but the use of dry powder inhaled antibiotics (DPIA) in these patients is extremely limited. This study seeks to analyse the efficacy and safety of DPIA in bronchiectasis patients. Material and methods: Multi-centre study of historic cohorts. All the hospital centres in Spain were contacted in order to collect data on patients with a diagnosis of bronchiectasis who had taken at least one dose of DPIA. Its efficacy was analysed in clinical, functional and microbiological terms by comparing the year before and the year after the prescription of DPIA. Adverse effects and variables associated with these effects, or any need to withdraw the drug, were also analysed. Results: 164 patients from 33 Spanish centres were included; 86% and 14% of these were treated with dry powder colistin and tobramycin, respectively. Chronic bronchial infection by Pseudomonas aeruginosa was present in 86% of these patients, and DPIA significantly reduced the number of exacerbations, the quantity and purulence of sputum and the isolation of pathogenic microorganisms. The most common adverse effect was cough (40%), particularly in cases of Chronic Obstructive Pulmonary Disease (COPD) and a previous cough and in those patients who had difficulties in handling the device. These factors were associated with a higher level of withdrawal of the treatment. There were no serious adverse effects. Conclusions: Our study suggests that DPIA are clinically efficacious and safe for treating bronchiectasis patients. Cough was shown to be the most common side-effect and reason for withdrawal of the treatment. Full article

(This article belongs to the Special Issue Cystic and Non-Cystic, Fibrosis Bronchiectasis: Drifting from Molecular to Clinical Practice)

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11 pages, 272 KiB

Open AccessArticle

Chronic Bacterial Infection Prevalence, Risk Factors, and Characteristics: A Bronchiectasis Population-Based Prospective Study

by Adelina Amorim, Leonor Meira, Margarida Redondo, Manuela Ribeiro, Ricardo Castro, Márcio Rodrigues, Natália Martins and Venceslau Hespanhol

J. Clin. Med. 2019, 8(3), 315; https://doi.org/10.3390/jcm8030315 - 6 Mar 2019

Cited by 2 | Viewed by 3161

Abstract

Background: Few data are available on chronic bacterial infections (CBI) in bronchiectasis patients. Given that CBI seems to trigger longer hospital stays, worse outcomes, and morbimortality, this study was undertaken to assess CBI prevalence, characteristics, and risk factors in outpatients with bronchiectasis. Methods: A total of 186 patients followed in a bronchiectasis tertiary referral centre in Portugal were included. Demographic data and information on aetiology, smoking history, mMRC score, Bronchiectasis Severity Index (BSI) score, sputum characteristics, lung function, exacerbations, and radiological involvement degree were collected. Results: Patients included (mean age 54.7 ± 16.2 years; 60.8% females) were followed up for a period of 3.8 ± 1.7 years. The most common cause of bronchiectasis was infection (31.7%) followed by immune deficiencies (11.8%), whereas in 29% of cases, no cause was identified. Haemophilus influenzae (32.3%) and Pseudomonas aeruginosa (30.1%) were the most common CBI-associated possible pathogenic microorganisms. CBI patients presented a higher follow-up time than no-CBI patients (p = 0.003), worse lung function, BSI (p < 0.001), and radiological (p < 0.001) scores, and more prominent daily sputum production (p = 0.002), estimated mean volume (p < 0.001), and purulent sputum (p < 0.001). The number of exacerbations/year (p = 0.001), including those requiring hospital admission (p = 0.009), were also higher in the CBI group. Independent CBI predictors were BSI score (OR 3.577, 95% CI 1.233–10.378), sputum characteristics (OR 3.306, 95% CI 1.107–9.874), and radiological score (OR 1.052, 95% CI 1.004–1.102). Conclusion: According to the CBI status, two different sub-groups of patients were found on the basis of several clinical outcomes, emphasizing the importance of routine sputum microbiological monitoring. Further studies are needed to better characterize CBI profiles and to define the individual clinical impact of the most prevalent pathogenic microorganisms. Full article

(This article belongs to the Special Issue Cystic and Non-Cystic, Fibrosis Bronchiectasis: Drifting from Molecular to Clinical Practice)

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32 pages, 519 KiB

Open AccessReview

Impact of Pseudomonas aeruginosa Infection on Patients with Chronic Inflammatory Airway Diseases

by Marta Garcia-Clemente, David de la Rosa, Luis Máiz, Rosa Girón, Marina Blanco, Casilda Olveira, Rafael Canton and Miguel Angel Martinez-García

J. Clin. Med. 2020, 9(12), 3800; https://doi.org/10.3390/jcm9123800 - 24 Nov 2020

Cited by 76 | Viewed by 6360

Abstract

Pseudomonas aeruginosa (P. aeruginosa) is a ubiquitous and opportunistic microorganism and is considered one of the most significant pathogens that produce chronic colonization and infection of the lower respiratory tract, especially in people with chronic inflammatory airway diseases such as asthma, chronic obstructive pulmonary disease (COPD), cystic fibrosis (CF), and bronchiectasis. From a microbiological viewpoint, the presence and persistence of P. aeruginosa over time are characterized by adaptation within the host that precludes any rapid, devastating injury to the host. Moreover, this microorganism usually develops antibiotic resistance, which is accelerated in chronic infections especially in those situations where the frequent use of antimicrobials facilitates the selection of “hypermutator P. aeruginosa strain”. This phenomenon has been observed in people with bronchiectasis, CF, and the “exacerbator” COPD phenotype. From a clinical point of view, a chronic bronchial infection of P. aeruginosa has been related to more severity and poor prognosis in people with CF, bronchiectasis, and probably in COPD, but little is known on the effect of this microorganism infection in people with asthma. The relationship between the impact and treatment of P. aeruginosa infection in people with airway diseases emerges as an important future challenge and it is the most important objective of this review. Full article

(This article belongs to the Special Issue Cystic and Non-Cystic, Fibrosis Bronchiectasis: Drifting from Molecular to Clinical Practice)

17 pages, 1801 KiB

Open AccessReview

The Efficacy of Therapeutic Respiratory Muscle Training Interventions in People with Bronchiectasis: A Systematic Review and Meta-Analysis

by Rocio Martín-Valero, Ana Maria Jimenez-Cebrian, Jose A Moral-Munoz, Maria de-la-Casa-Almeida, Manuel Rodriguez-Huguet and Maria Jesus Casuso-Holgado

J. Clin. Med. 2020, 9(1), 231; https://doi.org/10.3390/jcm9010231 - 15 Jan 2020

Cited by 14 | Viewed by 5107

Abstract

Background: Respiratory muscle dysfunction is an important health problem with high morbidity and mortality and associated costs in patients with bronchiectasis (BC). The aim of this study was to analyse the effects of therapeutic respiratory muscle training (RMT) interventions on improving sputum clearance, ventilator function, muscle strength and functional capacity in BC. Methods: Systematic review and meta-analysis were conducted following PRISMA guidelines. Two independent investigators searched using several electronic databases. The methodological quality of nine studies was assessed using the PEDro scale. Study selection/eligibility criteria: The following were included: randomised controlled trials, randomised crossover trials and pilot studies of patients with BC that used the intervention as RMT (inspiratory/expiratory) and evaluations of respiratory muscle strength (maximal expiratory pressure/maximal inspiratory pressure). This systematic review was registered in PROSPERO (CRD42017075101). Nine studies were included, five of which obtained an A recommendation grade, three with B, and one with C. Study quality was poor to good (mean PEDro Score of 6.375 out of 10). Studies had small sample sizes (8–98). Results show improvements on PImax in favour of therapeutic respiratory muscle training intervention (MD = 6.08; 95% CI = 1.38, 10.77; p < 0.01; I² = 92%). However, high heterogeneity was identified on meta-analysis. Full article

(This article belongs to the Special Issue Cystic and Non-Cystic, Fibrosis Bronchiectasis: Drifting from Molecular to Clinical Practice)

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27 pages, 427 KiB

Open AccessReview

Current Practices and Potential Nanotechnology Perspectives for Pain Related to Cystic Fibrosis

by Laura M. Trandafir, Magdalena M. Leon, Otilia Frasinariu, Ginel Baciu, Gianina Dodi and Elena Cojocaru

J. Clin. Med. 2019, 8(7), 1023; https://doi.org/10.3390/jcm8071023 - 12 Jul 2019

Cited by 8 | Viewed by 3959

Abstract

Pain is a complex, multidimensional process that negatively affects physical and mental functioning, clinical outcomes, quality of life, and productivity for cystic fibrosis (CF) patients. CF is an inherited multi-system disease that requires a complete approach in order to evaluate, monitor and treat patients. The landscape in CF care has changed significantly, with currently more adult patients than children worldwide. Despite the great advances in supportive care and in our understanding regarding its pathophysiology, there are still numerous aspects of CF pain that are not fully explained. This review aims to provide a critical overview of CF pain research that focuses on pain assessment, prevalence, characteristics, clinical association and the impact of pain in children and adults, along with innovative nanotechnology perspectives for CF management. Specifically, the paper evaluates the pain symptoms associated with CF and examines the relationship between pain symptoms and disease severity. The particularities of gastrointestinal, abdominal, musculoskeletal, pulmonary and chest pain, as well as pain associated with medical procedures are investigated in patients with CF. Disease-related pain is common for patients with CF, suggesting that pain assessment should be a routine part of their clinical care. A summary of the use of nanotechnology in CF and CF-related pain is also given. Further research is clearly needed to better understand the sources of pain and how to improve patients’ quality of life. Full article

(This article belongs to the Special Issue Cystic and Non-Cystic, Fibrosis Bronchiectasis: Drifting from Molecular to Clinical Practice)

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