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

Rationale: 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. Objectives: To investigate the prevalence of chronic obstructive pulmonary disease (COPD) associated with bronchiectasis and its relationship with clinical outcomes. We further investigated the impact of implementing the standardized ROSE criteria (radiological bronchiectasis [R], obstruction [FEV1/FVC ratio <0.7; O], symptoms [S], and exposure [⩾10 pack-years of smoking; E]), an objective definition of the association of bronchiectasis with COPD. Methods: Analysis of the EMBARC (European Bronchiectasis Registry), a prospective observational study of patients with computed tomography-confirmed bronchiectasis from 28 countries. The ROSE criteria were used to objectively define the association of bronchiectasis with COPD. Key outcomes during a maximum of 5 years of follow-up were exacerbations, hospitalization, and mortality. Measurements and Main Results: A total of 16,730 patients with bronchiectasis were included; 4,336 had a clinician-assigned codiagnosis of COPD, and these patients had more exacerbations, worse quality of life, and higher severity scores. We observed marked overdiagnosis of COPD: 22.2% of patients with a diagnosis of COPD did not have airflow obstruction and 31.9% did not have a history of ⩾10 pack-years of smoking. Therefore, 2,157 patients (55.4%) met the ROSE criteria for COPD. Compared with patients without COPD, patients who met the ROSE criteria had increased risks of exacerbations and exacerbations resulting in hospitalization during follow-up (incidence rate ratio, 1.25; 95% confidence interval, 1.15-1.35; vs. incidence rate ratio, 1.69; 95% confidence interval, 1.51-1.90, respectively). Conclusions: The label of COPD is often applied to patients with bronchiectasis who do not have objective evidence of airflow obstruction or a smoking history. Patients with a clinical label of COPD have worse clinical outcomes.

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. Adult patients with computed tomography-confirmed bronchiectasis were reviewed at baseline and annual follow-up visits using an electronic case report form. Asthma was diagnosed by the local investigator. Follow-up data were used to explore differences in exacerbation frequency between groups using a negative binomial regression model. Survival analysis used Cox proportional hazards regression. RESULTS: Of 16,963 patients with bronchiectasis included for analysis, 5,267 (31.0%) had investigator-reported asthma. Patients with BE+A were younger, were more likely to be female and never smokers, and had a higher body mass index than patients with bronchiectasis without asthma. BE+A was associated with a higher prevalence of rhinosinusitis and nasal polyps as well as eosinophilia and Aspergillus sensitization. BE+A had similar microbiology but significantly lower severity of disease using the bronchiectasis severity index. Patients with BE+A were at increased risk of exacerbation after adjustment for disease severity and multiple confounders. Inhaled corticosteroid (ICS) use was associated with reduced mortality in patients with BE+A (adjusted hazard ratio 0.78, 95% CI 0.63-0.95) and reduced risk of hospitalization (rate ratio 0.67, 95% CI 0.67-0.86) compared with control subjects without asthma and not receiving ICSs. CONCLUSIONS: BE+A was common and was associated with an increased risk of exacerbations and improved outcomes with ICS use. Unexpectedly we identified significantly lower mortality in patients with BE+A.

Airway clearance management in people with bronchiectasis: data from the European Bronchiectasis Registry (EMBARC).

BACKGROUND: International guidelines recommend airway clearance management as one of the important pillars of bronchiectasis treatment. However, the extent to which airway clearance is used for people with bronchiectasis in Europe is unclear. The aim of the study was to identify the use of airway clearance management in patients with bronchiectasis across different countries and factors influencing airway clearance use. METHODS: This was a prospective observational study using data from the European Multicentre Bronchiectasis Audit and Research Collaboration (EMBARC) Registry between January 2015 and April 2022. Prespecified options for airway clearance management were recorded, including airway clearance techniques, devices and use of mucoactive drugs. RESULTS: 16 723 people with bronchiectasis from 28 countries were included in the study. The mean age was 67 years (interquartile range 57-74 years, range 18-100 years) and 61% were female. 72% of the participants reported daily sputum expectoration and 52% (95% CI 51-53%) of all participants reported using regular airway clearance management. Active cycle of breathing technique was used by 28% of the participants and airway clearance devices by 16% of participants. The frequency of airway clearance management and techniques used varied significantly between different countries. Participants who used airway clearance management had greater disease severity and worse symptoms, including a higher daily sputum volume, compared to those who did not use it regularly. Mucoactive drugs were also more likely to be used in participants with more severe disease. Access to specialist respiratory physiotherapy was low throughout Europe, but particularly low in Eastern Europe. CONCLUSIONS: Only a half of people with bronchiectasis in Europe use airway clearance management. Use of and access to devices, mucoactive drugs and specialist chest physiotherapy appears to be limited in many European countries.

Objective sputum colour assessment and clinical outcomes in bronchiectasis: data from the European Bronchiectasis Registry (EMBARC).

BACKGROUND: A validated 4-point sputum colour chart can be used to objectively evaluate the levels of airway inflammation in bronchiectasis patients. In the European Bronchiectasis Registry (EMBARC), we tested whether sputum colour would be associated with disease severity and clinical outcomes. METHODS: We used a prospective, observational registry of adults with bronchiectasis conducted in 31 countries. Patients who did not produce spontaneous sputum were excluded from the analysis. The Murray sputum colour chart was used at baseline and at follow-up visits. Key outcomes were frequency of exacerbations, hospitalisations for severe exacerbations and mortality during up to 5-year follow-up. RESULTS: 13 484 patients were included in the analysis. More purulent sputum was associated with lower forced expiratory volume in 1 s (FEV1), worse quality of life, greater bacterial infection and a higher bronchiectasis severity index. Sputum colour was strongly associated with the risk of future exacerbations during follow-up. Compared to patients with mucoid sputum (reference group), patients with mucopurulent sputum experienced significantly more exacerbations (incident rate ratio (IRR) 1.29, 95% CI 1.22-1.38; p<0.0001), while the rates were even higher for patients with purulent (IRR 1.55, 95% CI 1.44-1.67; p<0.0001) and severely purulent sputum (IRR 1.91, 95% CI 1.52-2.39; p<0.0001). Hospitalisations for severe exacerbations were also associated with increasing sputum colour with rate ratios, compared to patients with mucoid sputum, of 1.41 (95% CI 1.29-1.56; p<0.0001), 1.98 (95% CI 1.77-2.21; p<0.0001) and 3.05 (95% CI 2.25-4.14; p<0.0001) for mucopurulent, purulent and severely purulent sputum, respectively. Mortality was significantly increased with increasing sputum purulence, hazard ratio 1.12 (95% CI 1.01-1.24; p=0.027), for each increment in sputum purulence. CONCLUSION: Sputum colour is a simple marker of disease severity and future risk of exacerbations, severe exacerbations and mortality in patients with bronchiectasis.

Nocardia spp. isolation in chronic lung diseases: Are there differences between patients with pulmonary nocardiosis and Nocardia colonization?

AIMS: Chronic lung diseases are a recognized risk factor for Nocardia spp. INFECTION: Nocardia spp. isolation does not inevitably imply disease, and thus colonization must be considered. Here, we aimed to analyse the differences between pulmonary nocardiosis (PN) and Nocardia spp. colonization in patients with chronic lung diseases. METHODS AND RESULTS: A retrospective study of patients with laboratory confirmation of isolation of Nocardia spp. in at least one respiratory sample was performed. Patients with PN and Nocardia spp. colonization were compared. There were 71 patients with Nocardia spp. identification, 64.8% were male, with a mean age of 67.7 ± 11.2 years. All patients had ≥1 pre-existing chronic lung disease, and 19.7% of patients were immunocompromised. PN and Nocardia spp. colonization were considered in 26.8% and 73.2% of patients, respectively. Symptoms and chest CT findings were significantly more frequent in patients with PN (p < 0.001). During follow-up time, 12 (16.9%) patients died, 6 in PN group. Immunosuppression, constitutional symptoms, haematological malignancy and PN diagnosis were associated with significantly shorter survival times, despite only immunosuppression (HR 3.399; 95% CI 1.052-10.989) and PN diagnosis (HR 3.568; 95% CI 1.078-11.910) remained associated with a higher death risk in multivariate analysis. CONCLUSIONS: PN was associated with clinical worsening, more chest CT findings and worse clinical outcomes. SIGNIFICANCE AND IMPACT OF STUDY: Nocardia spp. isolation in chronic lung disease patients is more common than expected and the differentiation between colonization and disease is crucial.

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. Data discussed here clearly denote the rarity, non-specificity and heterogeneity of clinical presentations of the disease, reinforcing the need for individualized therapeutic approaches.

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. We recruited 134 patients (median[interquartile range] 16[11-22.25]y; 54% male; 31% cystic fibrosis, 29% other respiratory diseases, 28% asthma; 12% no respiratory diseases) at the Pediatrics and Pulmonology departments of a tertiary hospital. First, clinicians performed conventional auscultation with analog stethoscopes at 4 locations (trachea, right anterior chest, right and left lung bases), and documented any adventitious sounds. Then, smartphone auscultation was recorded twice in the same four locations. The recordings (n = 1060) were classified by two annotators. Seventy-three percent of recordings had quality (obtained in 92% of the participants), with the quality proportion being higher at the trachea (82%) and in the children's group (75%). Adventitious sounds were present in only 35% of the participants and 14% of the recordings, which may have contributed to the fair agreement between conventional and smartphone auscultation (85%; k = 0.35(95% CI 0.26-0.44)). Our results show that smartphone auscultation was feasible, but further investigation is required to improve its agreement with conventional auscultation.

[The Impact of CFTR Modulating Therapy on Chronic Lung Infection in Patients with Cystic Fibrosis]. / O Impacto da Terapêutica Moduladora da CFTR na Infeção Pulmonar Crónica em Doentes com Fibrose Quística.

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. These changes culminate in inflammation, chronic pulmonary infection, and recurrent exacerbations, with lung disease being the main cause of morbidity and mortality. In the early stages of the disease, Staphylococcus aureus is generally the agent responsible for chronic infection. Over time, Pseudomonas aeruginosa becomes more prevalent, being the most frequent bacteria in adults. However, in up to 70% of patients, colonization is polymicrobial, with frequent isolation of S. aureus and P. aeruginosa, associated with Haemophilus influenzae or Streptococcus pneumoniae, as well as isolation of other bacterial agents, viruses, or fungi. In recent years, drugs modulating CFTR have been developed which have shown a positive effect on lung function, body mass index, exacerbation rate, chlorine concentration, and quality of life. Currently, four drugs are approved that act by improving the function or increasing the amount of protein produced and consequently the ion transport. [...].

A fibrose quística é a doença genética letal mais comum na população branca, afetando aproximadamente 80 000 pessoas em todo o mundo. É uma doença autossómica recessiva, monogenética e multissistémica, estando descritas mais de 2000 mutações no gene da proteína CFTR. A disfunção desta proteína leva à diminuição da secreção de cloro e de bicarbonato, hiperabsorção de sódio e consequentemente de água, resultando no espessamento das secreções e acumulação de agentes patogénicos. Estas alterações culminam em inflamação, infeção pulmonar crónica e agudizações recorrentes, sendo a doença pulmonar a principal causa de morbilidade e mortalidade. Nas fases iniciais da doença, o Staphylococcus aureus é, geralmente, o agente responsável pela infeção crónica. Com o tempo, a Pseudomonas aeruginosa vai adquirindo um papel mais preponderante, sendo a bactéria mais frequente nos adultos. Contudo, em até 70% dos doentes, a colonização é polimicrobiana, sendo frequente o isolamento de S. aureus e P. aeruginosa, associado a Haemophilus influenzae ou Streptococcus pneumoniae, bem como o isolamento de outros agentes bacterianos, vírus ou fungos. Nos últimos anos foram desenvolvidos fármacos moduladores da CFTR, que demonstraram efeito positivo na função pulmonar, índice de massa corporal, taxa de exacerbações, concentração de cloro e qualidade de vida. Atualmente, estão aprovados quatro fármacos que atuam melhorando a função ou aumentando a quantidade de proteína produzida e consequentemente o transporte dos iões. [...].

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. In the first one, a task force of international experts derived from published guidelines and graded ten quality standards for adult CF care using a modified Delphi methodology. In the second phase, an international audit was conducted among adult CF centers to retrospectively validate the quality statements and monitor adherence. RESULTS: The task force identified 10 quality standards specific to the care of adults with CF, mainly based on the 2018 ECFS standards of care. 14 adult CF centers participated in the audit, which showed that most quality standards for the management of CF in adults are met across Europe. Heterogeneity in adherence to standards was found across centers according to geographical setting and centers' characteristics. CONCLUSIONS: The identification of quality standards is a valuable resource for the standardization and monitoring of care delivery across centers taking care of adults with CF.

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. Clinical status, laboratory parametres, nutrition, lung function tests, genetics and bone mineral density (BMD) data were collected from a CF reference centre. RESULTS: Of 30 patients, 53.3% were males (n=16). Median age was 32.5 (27.0; 32,5) and median body mass index (BMI) was 22,04 (19,85; 24,55), with 4 patients (13.3%) being underweight (BMI<18.5 kg/m²). Four patients (13.3 %) were diagnosed with osteoporosis and 15 patients (50%) has low BMD. Among them, 2 (6.7%) had fragility fractures. A moderate correlation was found between the lumbar spine (LS) BMD and BMI and, as expected, femural neck BMD and LS BMD has moderate to strong correlations with BMD Z scores. CONCLUSION: Despite the young middle age we found a high prevalence of low BMD and osteoporosis in patients with CF. Early recognition and treatment are the most effective strategies for reducing the morbidity due to osteoporosis in these patients.

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. CASE DESCRIPTION: A 44 -year -old woman with pulmonary tuberculosis in childhood and with the diagnosis of bilateral extensive bronchiectasis who presented behaviour alterations and later, paresis of the sixth cranial nerve, was diagnosed multiple brain abscesses. The microbiological exams were negative. The study of the primary focus of infection could only identify infected bronchiectasis. Empiric antibiotics and anti- -oedematous treatment were prescribed with progressive clinical improvement. Because of inadequate response she was submitted to surgery. She was discharged with diplopia, without any other neurological alterations. CONCLUSION: In the present clinical case, the infected bronchiectasis were the only focal infection detected, so in patients with this disease and with new neurological manifestations, infected lesions in the central nervous system should be excluded.

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. 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.

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. Following a systematic review, the panel used a modified Delphi process with a combination of face-to-face meetings and e-surveys to develop a definition that can be used in research settings for children and adults with PCD. A pulmonary exacerbation was defined by the presence of three or more of the following seven items: 1) increased cough, 2) change in sputum volume and/or colour, 3) increased shortness of breath perceived by the patient or parent, 4) decision to start or change antibiotic treatment because of perceived pulmonary symptoms, 5) malaise, tiredness, fatigue or lethargy, 6) new or increased haemoptysis, and 7) temperature >38°C. The consensus panel proposed that the definition should be used for future clinical trials. The definition should be validated and the usability assessed during these studies.

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. Pneumonia Severity Index (PSI) and British Thoracic Society (BTS) CURB-65 scoring tools were evaluated. The statistical treatment was performed using the SPSS 14.0 program. We included 104 patients, 67.3% male, median age 63 years old, mortality 13.4%. There was a significant association between the PSI and CURB-65 score and mortality. Despite advances, CAP is still an important health problem with a high attendant morbi-mortality. This study confirms the value of PSI and CURB-65 in the prediction of severe pneumonia.

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. Molecular analyses were conducted in clinical isolates by testing the newly colony-PCR strategy and SNaPaer assay. A total of 207 isolates of P. aeruginosa were collected from clinical samples. To assess the assay specificity, other Gram-negative non-aeruginosa bacteria, namely Pseudomonas and Burkholderia, were tested. The complete group of 23 markers included in the SNaPaer panel was observed exclusively in P. aeruginosa; more than 18 markers failed in other bacteria. A total of 43 SnaP profiles were obtained for clinical P. aeruginosa, being the profiles highly patient-specific. Six CF patients were colonized with P. aeruginosa isolates with very distinct SnaP profiles, particularly following adjustments on antibiotic therapy, thus suggesting changes on the dynamics and dominance of these bacteria. SnaPaer proved to be a good and reliable tool for identification and genotyping of clinical P. aeruginosa in a single-tube multiplex PCR. Combined with the proposed colony-PCR strategy, SnaPaer assay facilitates the molecular analysis of P. aeruginosa.

[Pleural disease and acquired immunodeficiency syndrome]. / Doença pleural e síndroma de imunodeficiência adquirida.

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. The total incidence of pneumothorax (PTX) in patients with acquired immune deficiency syndrome (AIDS) varies from 2.7% to 4.9%. The great majority occurs in patients with current or previous Pneumocystis carinii infection, who present subpleural pulmonary cavities with necrosis. The treatment of spontaneous PTX in patients with AIDS is difficult, with an increased tendency to bronchopleural fistula persistence. The use of tube thoracostomy, with or without pleural sclerose, can be insufficient to resolve PTX. Other therapeutic options are attachment of a Heimlich valve or surgical intervention. The prevalence and the etiology of pleural effusion (PE) among hospitalized patients with AIDS varies widely. One reason that can contribute to this variability is the difference on risk factors associated with HIV infection, in the studied population. Parapneumonic effusions, tuberculosis and Kaposi's sarcoma are the most common causes. Empyemas are a rare pleural complication. Although Pneumocystis carinii pneumonia is a common cause of pneumonias in AIDS patients, it is an unusual cause of pleural effusion. Other possible causes of pleural effusion are non-Hodgkin's lymphoma, namely body cavity-based lymphoma.

[Endobronchial tuberculosis -- clinical and bronchoscopic features]. / Tuberculose endobrônquica -- alterações clínicas e broncoscópicas.

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. There were 8 (57%) men and 6 women with a median age of 39.6 +/- 18.1 years (range from 20 to 78 years). Cough was the most common complain and it was present in 71.4% of patients. Only 5 patients were sputum smear positive. Five patients (35.7%) had parenchymal infiltration and this was the most common roentgenographic appearance. Forms of ET were classified into subtypes: actively caseating (n=4), granular (n=3), tumorous (n=3), edematous-hyperemic (n=2) and ulcerative (n=2). The upper lobes were affected in 9 (64.3%) patients. Nine patients had involvement of the left bronchial tree, 3 of the right and in 2 there were bilateral lesions. The diagnosis could be established in 11 (78.6%) cases by bronchial biopsy. All patients had positive bronchial lavage cultures for acid-fast bacilli. Clinical manifestations and roentgenographic appearance of ET are not specific and so bronchoscopy is mandatory for the prompt diagnosis and follow-up of its evolution.

[Home cardiorespiratory sleep study in children. Will it be feasible?]. / Estudo cardio-respiratório do sono domiciliário em crianças. Será exequível?

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. It was our aim to study the yield of the home cardiorespiratory sleep studies carried out in children. Since January of 1999 until June of 2003, 33 home cardiorespiratory sleep studies were performed in children. We studied 31 children (21 male) with a median age of 10.6+/-3.4 years. Five children had craniofacial malformations, 2 neuromuscular diseases and 10 were obese. The signals of nasal flow and saturation were good/acceptable in 67.7% and 96.8% of the cases, respectively. In 2 cases the register was null. Average of apnea-hypopnea index was of 10.7+/-12.3/hour, average saturation of 95.6%+/-3.0 %, minimum saturation of 82.2 %+/-9.2% and dessaturation index of 12.5+/-10.7/hour. Childhood obstructive sleep apnea syndrome was confirmed/suggested in 30 children. The apnea-hypopnea index and the dessaturation index were significantly higher in the group of children with craniofacial malformations and neuromuscular disorders comparatively to children with obesity (26.3 versus 10.5 and 21.5 versus 11.3, respectively) but without statistical significance. In our experience, home cardiorespiratory sleep studies is a diagnostic method easily used in children. This method gives more information comparatively to other screening techniques so that it can evaluate with more accuracy the existence of sleep disordered breathing and may be a possible alternative to polysomnography.

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. A simple and informative multiplex, SNaPaer assay, was developed and genotyping of Pseudomonas aeruginosa was obtained after a single reaction of multiplex PCR amplification and mini-sequencing. This cost-effective technique allowed the analysis of a Portuguese set of isolates (n = 111) collected from three distinct hospitals and the genotyping data could be obtained in less than six hours. Point mutations were shown to be the most frequent event responsible for diversification of the Portuguese population sample. The Portuguese isolates corroborated the epidemic hypothesis for P. aeruginosa population. SNaPaer genotyping assay provided a discriminatory power of 0.9993 for P. aeruginosa, by testing in silico several hundreds of MLST profiles available online. The newly proposed assay targets less than 0.01% of the total MLST length and guarantees reproducibility, unambiguous analysis and the possibility of comparing and transferring data between different laboratories. The plasticity of the method still supports the addition of extra molecular markers targeting specific purposes/populations. SNaPaer can be of great value to clinical laboratories by facilitating routine genotyping of P. aeruginosa.

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. PF, ABG, and 6MWD were evaluated before and after PR to determine the potential influence of gender, exacerbations, underlying cause of bronchiectasis, severity of obstruction, and colonization with bacteria. RESULTS: Forty-one patients (48.8% males; median age, 54 years) were included; 25 had severe obstruction and 19 were colonized with bacteria. Following PR, no significant changes were detected in PF or ABG. Median 6MWD before PR was 425 m and post-PR was 450 m (P = .431). Outcomes did not show any interaction with gender, colonization, or exacerbations. However, patients with idiopathic bronchiectasis did show a significant improvement in forced vital capacity in percent of predicted and residual volume after PR (P = .016 and .048, respectively). Patients with severe obstruction showed a statistically significant decrease in percent of predicted residual volume (P = .025). CONCLUSION: There appears to be a beneficial impact of PR on PF in certain groups of patients with bronchiectasis. In addition, PR indications and protocols for patients with bronchiectasis may need to be adapted to accommodate specific patients, so that expressive exercise capacity improvement can be achieved.