Bronchiectasis and asthma: Data from the European Bronchiectasis Registry (EMBARC).

Background: Asthma is commonly reported in patients with a diagnosis of bronchiectasis.

Objective: The aim of this study was to evaluate whether patients with bronchiectasis and asthma (BE+A) had a different clinical phenotype and different outcomes compared with patients with bronchiectasis without concomitant asthma.

Methods: A prospective observational pan-European registry (European Multicentre Bronchiectasis Audit and Research Collaboration) enrolled patients across 28 countries.

The Association between Bronchiectasis and Chronic Obstructive Pulmonary Disease: Data from the European Bronchiectasis Registry (EMBARC).

COPD and bronchiectasis are commonly reported together. Studies report varying impacts of co-diagnosis on outcomes, which may be related to different definitions of disease used across studies. To investigate the prevalence of chronic obstructive pulmonary disease (COPD) associated with bronchiectasis and its relationship with clinical outcomes.

Monitoring of ECFS quality standards for the clinical management of adults with cystic fibrosis.

Background: Although cystic fibrosis (CF) standards of care have been produced and regularly updated, they are not specifically targeting at the adult population. The ECFS Standards of Care Project established an international task force of experts to identify quality standards for adults with CF and assess their adherence.

Methods: This study was composed of two phases.

[The Impact of CFTR Modulating Therapy on Chronic Lung Infection in Patients with Cystic Fibrosis].

Cystic fibrosis is the most common lethal genetic disease in the white population, affecting approximately 80 000 people worldwide. It is an autosomal recessive, monogenic, and multisystemic disease, with over 2000 mutations described in the CFTR protein gene. The dysfunction of this protein leads to a decrease in the secretion of chlorine and bicarbonate, sodium hyperabsorption, and consequent water absorption, resulting in the thickening of secretions and accumulation of pathogens.

Bone involvement in young adults with cystic fibrosis - a Portuguese cohort.

Introduction: Long-term survivors of cystic fibrosis (CF) have a dramatic increase in the risk of osteoporosis and incident fracture. The objective of this work is to characterize a CF related bone disease in a Portuguese cohort of CF patients.

Methods: We performed a cross-sectional, observational study on a cohort of CF adult patients.

Enlightening clinical and therapeutic heterogeneity in thoracic actinomycosis: A review in bronchiectasis and non-bronchiectasis patients.

Actinomycosis is a rare chronic infection triggered by species of Actinomyces. Although thoracic involvement represents about 15% of human actinomycosis, its true incidence may be underestimated, not only because of its challenging diagnosis, but also because it can be treated unintentionally with antibiotics for other diseases. In this sense, this work aims at providing an up-to-date literature review on thoracic actinomycoses, with particular emphasis on presentation, diagnostic and therapeutic approaches, also paving upcoming clinical interventions from findings obtained of a presentation of a case series.

Lung Auscultation Using the Smartphone-Feasibility Study in Real-World Clinical Practice.

Conventional lung auscultation is essential in the management of respiratory diseases. However, detecting adventitious sounds outside medical facilities remains challenging. We assessed the feasibility of lung auscultation using the smartphone built-in microphone in real-world clinical practice.

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

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.

Pulmonary exacerbations in patients with primary ciliary dyskinesia: an expert consensus definition for use in clinical trials.

Pulmonary exacerbations are a cause of significant morbidity in patients with primary ciliary dyskinesia (PCD) and are frequently used as an outcome measure in clinical research into chronic lung diseases. So far, there has been no consensus on the definition of pulmonary exacerbations in PCD. 30 multidisciplinary experts and patients developed a consensus definition for children and adults with PCD.

Molecular identification and genotyping of Pseudomonas aeruginosa isolated from cystic fibrosis and non-cystic fibrosis patients with bronchiectasis.

There is no standard methodology for the molecular identification and genotyping of Pseudomonas aeruginosa which are frequently isolated in bronchiectasis patients. Hence, the main goal of this work was to propose a methodology capable to simultaneously identify and genotype, in less than 6 h, clinical P. aeruginosa collected from cystic fibrosis (CF) and non-CF patients with bronchiectasis.

SNaPaer: a practical single nucleotide polymorphism multiplex assay for genotyping of Pseudomonas aeruginosa.

Multilocus sequence typing (MLST) represents the gold standard genotyping method in studies concerning microbial population structure, being particularly helpful in the detection of clonal relatedness. However, its applicability on large-scale genotyping is limited due to the high cost and time spent on the task. The selection of the most informative nucleotide positions simplifies genomic characterization of bacteria.

Pulmonary rehabilitation in patients with bronchiectasis: pulmonary function, arterial blood gases, and the 6-minute walk test.

Background: Information regarding the effects of pulmonary rehabilitation (PR) on pulmonary function (PF), arterial blood gases (ABG), and 6-minute walk distance (6MWD) in patients with bronchiectasis is scant in the literature.

Methods: To evaluate the effects of PR on these indices in this population, a retrospective evaluation of those who attended PR from 2007 to 2010, was made. Pulmonary rehabilitation lasted a mean of 12 weeks and included cycle ergometer exercise for 30 minutes, 3 times per week, with additional upper limbs and quadriceps training.

Susceptibility to five antifungals of Aspergillus fumigatus strains isolated from chronically colonised cystic fibrosis patients receiving azole therapy.

Exposure of Aspergillus fumigatus to stressful antifungal therapies may result in decreased susceptibility. The aim of the present work was to evaluate the susceptibility to azole and non-azole antifungals of 159 isolates of A. fumigatus collected from cystic fibrosis (CF) patients receiving azole antifungal therapy.

Multiple brain abscesses - a rare complication of bronchiectasis.

Background: Brain abscess can arise as a complication of a variety of infections, trauma or surgery. Bacteria can invade the brain by direct spread or through haematogenous seeding. Brain abscesses are described as a rare complication of bronchiectasis.

Streptococcus pneumoniae - caused CAP in hospitalised patients: mortality predictors.

Probably the most important decision in the management of Community-Acquired Pneumonia (CAP) is patient site of care. Patients with Streptococcus pneumoniae-caused CAP admitted to our hospital between 1st January and 31st December 2006 were retrospectively analysed. Samples of blood, sputum, bronchial and bronchoalveolar lavage and urine were collected for microbiological testing using standard culture techniques and urine antigen detection.

[Home cardiorespiratory sleep study in children. Will it be feasible?].

Childhood obstructive sleep apnea syndrome is a common condition and can result in serious complications. The nocturnal polysomnography remains the gold standard in the diagnosis of this pathology. Given the scarcity of sleep laboratories, namely with paediatric profile, screening techniques have been commonly used.

[Endobronchial tuberculosis -- clinical and bronchoscopic features].

Endobronchial tuberculosis (ET) is a serious complication of pulmonary tuberculosis and is a major cause of morbidity. The aim of our retrospective study was to characterize the clinical, radiological, microbiological and bronchoscopic features of ET. Between January 1999 and June 2002 a total of 14 patients were diagnosed as having ET in our hospital.

[Pleural disease and acquired immunodeficiency syndrome].

Respiratory infections are among the most common complications in patients infected with human immune deficiency virus (HIV) and can occur at all CD4 level. Pleural complications are uncommon but they have some distinctive aspects from HIV-negative patients. The PTX occurrence in HIV-positive patients was described for the first time in 1984.