Quality of Life Questionnaire-Bronchiectasis: a study of the psychometric properties of the Brazilian Portuguese version.

OBJECTIVE: To evaluate the psychometric properties of the Brazilian Portuguese version of the Quality of Life Questionnaire-Bronchiectasis. DESIGN: Cross-sectional study. SETTING: Outpatient clinic. SUBJECTS: Clinically stable individuals with a diagnosis of bronchiectasis. MEASURES: The evaluations performed were spirometry, incremental shuttle walk test, Saint George's Respiratory Questionnaire, and the modified Medical Research Council dyspnea scale. The Quality of Life Questionnaire-Bronchiectasis was administered twice (seven to 14 days apart). Psychometric analyses were performed as follows: reliability, construct validity, criterion validity, and interpretability. RESULTS: In total, 108 individuals (48 ± 14 years, 61 women) participated in the study. Internal consistency was considered adequate (Cronbach's alpha ⩾ 0.70) for the majority of scales (from 0.58 to 0.93). Test-retest coefficients were moderate to excellent (intraclass correlation coefficients from 0.70 to 0.93). In the construct validity, 35 of 37 items correlated more strongly with their assigned scale than a competing scale. The convergent validity showed significant correlations between scales of the Quality of Life Questionnaire-Bronchiectasis with modified Medical Research Council dyspnea scale, and incremental shuttle walk test (r from 0.20 to 0.59). A low to moderate correlations was revealed between all scales of the Quality of Life Questionnaire-Bronchiectasis and the Saint George's Respiratory Questionnaire domains (r from 0.26 to 0.70). The standard error of measurement was acceptable. Ceiling effects were found for the Social Functioning and Treatment Burden scales. CONCLUSIONS: The Quality of Life Questionnaire-Bronchiectasis is a reliable, valid instrument with adequate internal consistency for the evaluation of the impact of bronchiectasis on the health-related quality of life of Brazilian adults.

COPD Assessment Test (CAT) is a Valid and Simple Tool to Measure the Impact of Bronchiectasis on Affected Patients.

The COPD assessment test (CAT) is a short questionnaire developed to help patients and clinicians to assess the impact of symptoms in routine clinical practice. We aimed to validate and to test the reproducibility of CAT in patients with bronchiectasis and correlate with the severity of dyspnea, aerobic and functional capacity, and physical activity in daily life. This is a cross-sectional study, patients with bronchiectasis underwent spirometry, cardiopulmonary exercise test (CPET), incremental shuttle walk test (ISWT), Saint George`s Respiratory Questionnaire (SGRQ), and received pedometer. CAT was applied twice (CAT-1 and CAT-2, 7 to 10 days apart). The severity of bronchiectasis was assessed by E-FACED and bronchiectasis severity index (BSI). A total of 100 patients were evaluated (48 ± 14 years, 59 women, FVC: 67 ± 22% pred, FEV1: 52 ± 25% pred). According to CAT, 14% patients presented low, 40% medium, 32% high, and 14% very high impact. The higher the CAT, the worse the severity of bronchiectasis, dyspnea, quality of life, performance on the CPET, and smaller the distance walked (DW) on the ISWT and number of steps (NS) per day. There was significant correlation between CAT and SGRQ, E-FACED, BSI, NS, ISWT, oxygen uptake, and workload at CPET. CAT-1 and CAT-2 presented similar values: 21 (13-26) and 19 (13-26), respectively. The CAT is a valid and reproducible instrument in patients with bronchiectasis presenting good correlation with clinical, functional, and quality of life measurements. This easy-to-use, easy-to-understand, quick, and useful tool may play an important role to assess the impact of bronchiectasis on both daily medical practice and clinical trial settings.

Latin America validation of FACED score in patients with bronchiectasis: an analysis of six cohorts.

BACKGROUND: The FACED score is an easy-to-use multidimensional grading system that has demonstrated an excellent prognostic value for mortality in patients with bronchiectasis. A Spanish group developed the score but no multicenter international validation has yet been published. METHODS: Retrospective and multicenter study conducted in six historical cohorts of patients from Latin America including 651 patients with bronchiectasis. Clinical, microbiological, functional, and radiological variables were collected, following the same criteria used in the original FACED score study. The vital status of all patients was determined in the fifth year of follow-up. The area under ROC curve (AUC-ROC) was used to calculate the predictive power of the FACED score for all-cause and respiratory deaths and both number and severity of exacerbations. The discriminatory power to divide patients into three groups of increasing severity was also analyzed. RESULTS: Mean (SD) age of 48.2 (16), 32.9% of males. The mean FACED score was 2.35 (1.68). During the follow up, 95 patients (14.6%) died (66% from respiratory causes). The AUC ROC to predict all-cause and respiratory mortality were 0.81 (95% CI: 0.77 to 0.85) 0.84 (95% CI: 0.80 to 0.88) respectively, and 0.82 (95% CI: 078-0.87) for at least one hospitalization per year. The division into three score groups separated bronchiectasis into distinct mortality groups (mild: 3.7%; moderate: 20.7% and severe: 48.5% mortality; p < 0.001). CONCLUSIONS: The FACED score was confirmed as an excellent predictor of all-cause and respiratory mortality and severe exacerbations, as well as having excellent discriminative capacity for different degrees of severity in various bronchiectasis populations.

Effects of periodontal treatment on exacerbation frequency and lung function in patients with chronic periodontitis: study protocol of a 1-year randomized controlled trial.

BACKGROUND: Chronic obstructive pulmonary disease (COPD) has been associated with periodontal disease (PD), and periodontal treatment (PT) has been connected to reduction of lung disease exacerbations. Bronchiectasis has many clinical similarities with COPD but, although it is also a chronic lung disease, to date it has not been studied with relation to PD. The aim of this study is to evaluate whether PT associated with photodynamic therapy (PDT) reduces the number of exacerbations, improves pulmonary function, periodontal clinical parameters and quality of life after 1 year of periodontal treatment follow-up. METHODS: Bronchiectasis patients will undergo medical anamnesis and periodontal examination. Participants with periodontitis will be divided into two groups and PT will be performed as G1 control group (n = 32) - OHO (oral hygiene orientation) + supragingival treatment + simulation of using photodynamic therapy (PDT); G2 experimental (n = 32) - scaling and root planing + PDT + OHO. Lung function will be assessed both at baseline and after 1 year by spirometry, exacerbation history will be analyzed through clinical records monitoring. Three instruments for quality of life assessment will also be applied - Saint George's Respiratory Questionnaire and Impact Profile Analysis Oral health (OHIP-14). It is expected that periodontal treatment can improve the analyzed parameters after 1 year. DISCUSSION: Although only one study evaluates exacerbation in COPD after 1 year of PT, bronchiectasis has not been studied in the dentistry field to date. TRIAL REGISTRATION: NCT02514226. Version #1. This study protocol receives grant from FAPESP (São Paulo Research Foundation) #2015/20535-1. First received: July 22, 2015, 1st version. This protocol has been approved by the Research Ethics Committee of Nove de Julho University.

Can severe asthmatic patients achieve asthma control? A systematic approach in patients with difficult to control asthma followed in a specialized clinic.

BACKGROUND: Despite advances in asthma treatment, severe asthma (SA) still results in high morbidity and use of health resources. Our hypothesis was that SA patients would achieve adequate control with a systematic protocol, including oral corticosteroids, budesonide/formoterol maintenance and reliever therapy and a multidisciplinary approach to improve adherence. METHODS: Non-controlled (NC) SA patients were enrolled to receive 2 weeks of oral corticosteroids and 12 weeks of formoterol + budesonide. Assessments included asthma control questionnaire (ACQ), asthma control test (ACT), daily symptom diary, lung function and health-related quality of life (HRQoL) questionnaires. RESULTS: Of 51 patients, 13 (25.5%) achieved control. NC patients had higher utilization of health resources and higher exacerbation rates. Both controlled (C) and NC patients had significantly reduced ACQ scores after oral corticosteroid treatment. After 12 weeks, C patients continued improving. NC patients did not have significant changes. A similar pattern was found regarding lung function, use of rescue medication, and days free of symptoms. After 2 weeks of oral corticosteroids, an increase occurred in those who achieved the ACQ cut off; however, 53.8% of C patients had an ACQ < 1.57 versus 21.1% of NC patients (p = 0.03). Both groups had low HRQoL at baseline with improvement after intervention. CONCLUSIONS: Despite rigorous, optimized follow-up treatment, 75% of SA patients did not achieve adequate symptom control and presented with impaired quality of life. Conversely, application of a low-cost, easy to implement systematic protocol can prevent up to 25% of SA patients from up-titrating to new and complex therapies, thus reducing costs and morbidity. TRIAL REGISTRATION: Retrospectively registered at ClinicalTrial.gov on 22 February 2010 ( NCT01089322 ).

Incremental shuttle walking test: a reproducible and valid test to evaluate exercise tolerance in adults with noncystic fibrosis bronchiectasis.

OBJECTIVE: To analyze the reliability, validity, and determinants of the incremental shuttle walk test (ISWT) in adults with noncystic fibrosis bronchiectasis. DESIGN: Cross-sectional study. SETTING: Outpatient clinic. PARTICIPANTS: Subjects (N=75; 26 men) underwent, on different days, cardiopulmonary exercise testing (CPET) and 2 ISWTs, 30 minutes apart. The number of steps in daily life was recorded. Concurrent validity was tested by the relation between distance walked with peak load and oxygen consumption (V˙o2). INTERVENTIONS: None. MAIN OUTCOME MEASURES: Distance walked (m) was compared between the first and second ISWTs; greatest distance walked was correlated with peak load and Vo2peak obtained from CPET, steps per day, and dyspnea evaluated by the Medical Research Council (MRC) scale; and desaturation was compared between CPET and the ISWT. RESULTS: Distance walked was equivalent between the first ISWT (441±152m) and the second ISWT (445±153m) with an excellent intraclass correlation coefficient (.995; 95% confidence interval, .99-.997). There were significant correlations between distance walked and peak load (r=.82), V˙o2 (r=.72), steps per day (r=.61), and the MRC scale (r=-.69). Age, body mass index, sex, forced vital capacity (% predicted), dyspnea, and steps per day explained 70% of the variation in distance walked (m) and 60% of the variance when expressed as percent predicted. Higher desaturation was observed during the ISWT (-4%±4%) than cycling (-2±3%) (P<.001). CONCLUSIONS: The ISWT is reliable, represents functional capacity, and induces greater desaturation than cycling. Age, body composition, pulmonary function, dyspnea, and physical activity in daily life are determinants of the distance walked on the ISWT.

Brazilian guidelines for the pharmacological treatment of the pulmonary symptoms of cystic fibrosis. Official document of the Sociedade Brasileira de Pneumologia e Tisiologia (SBPT, Brazilian Thoracic Association).

Cystic fibrosis (CF) is a genetic disease that results in dysfunction of the CF transmembrane conductance regulator (CFTR) protein, which is a chloride and bicarbonate channel expressed in the apical portion of epithelial cells of various organs. Dysfunction of that protein results in diverse clinical manifestations, primarily involving the respiratory and gastrointestinal systems, impairing quality of life and reducing life expectancy. Although CF is still an incurable pathology, the therapeutic and prognostic perspectives are now totally different and much more favorable. The purpose of these guidelines is to define evidence-based recommendations regarding the use of pharmacological agents in the treatment of the pulmonary symptoms of CF in Brazil. Questions in the Patients of interest, Intervention to be studied, Comparison of interventions, and Outcome of interest (PICO) format were employed to address aspects related to the use of modulators of this protein (ivacaftor, lumacaftor+ivacaftor, and tezacaftor+ivacaftor), use of dornase alfa, eradication therapy and chronic suppression of Pseudomonas aeruginosa, and eradication of methicillin-resistant Staphylococcus aureus and Burkholderia cepacia complex. To formulate the PICO questions, a group of Brazilian specialists was assembled and a systematic review was carried out on the themes, with meta-analysis when applicable. The results obtained were analyzed in terms of the strength of the evidence compiled, the recommendations being devised by employing the GRADE approach. We believe that these guidelines represent a major advance to be incorporated into the approach to patients with CF, mainly aiming to favor the management of the disease, and could become an auxiliary tool in the definition of public policies related to CF.

Home-based pulmonary rehabilitation in people with bronchiectasis: a randomised controlled trial.

AIM: To investigate the short- and long-term effects of home-based pulmonary rehabilitation (HBPR) on functional capacity, quality of life, peripheral muscle strength, dyspnoea and daily physical activity in people with bronchiectasis. METHODS: Randomised controlled trial with 63 participants with bronchiectasis. The HBPR group performed three sessions per week for 8 weeks (aerobic exercise: step training for 20 min; resistance training: exercises for quadriceps, hamstrings, deltoids and biceps brachii using elastic bands). The control group received a recommendation to walk at moderate intensity, three times per week. A weekly phone call was conducted for all participants, and the HBPR group received a home visit every 15 days. The primary outcome was distance in the incremental shuttle walk test (ISWT). Secondary outcomes were time in the endurance shuttle walk test (ESWT), number of steps in the incremental step test, quality of life, quadriceps muscle strength and daily physical activity. Measures were taken before and after intervention and 6 months later. RESULTS: After the intervention, the HBPR group had increased ISWT distance compared with the control group with between-group difference 87.9 m (95% CI 32.4-143.5 m). In addition, between-group differences were found in the ESWT, incremental step test, quality of life and quadriceps muscle strength, favouring the HBPR group. After 6 months, no differences were observed between the groups. CONCLUSION: HBPR is an effective alternative offering of pulmonary rehabilitation for people with bronchiectasis. However, the programme was not effective in maintaining the benefits after 6 months of follow-up.

Systemic Inflammation and Oxidative Stress in Adults with Bronchiectasis: Association with Clinical and Functional Features.

OBJECTIVES: To compare the inflammatory and oxidative stress (OS) states of adults with bronchiectasis with those of healthy controls and correlate inflammatory and OS levels with lung function and physical capacity. METHODS: This study used a cross-sectional design. Seventy-four adults with bronchiectasis (age: 49±15 years, forced expiratory volume in 1 second [FEV1]: 52.5±25.6%) and 42 healthy controls (age: 44±17 years, FEV1: 95.9±14.0%) performed cardiopulmonary exercise tests and incremental shuttle walking tests. Their physical activity in daily life, inflammatory cytokine, and antioxidant levels in plasma were measured. RESULTS: Compared to that of the controls, the levels of interleukin (IL)-6 (p<0.001), IL-10 (p<0.001), carbonylated proteins (p=0.001), and superoxide anions (p=0.046) were significantly increased in adults with bronchiectasis. Catalase activity was also reduced in this group (p<0.001). The inflammatory markers IL-1ß, IL-6, and tumor necrosis factor-α correlated negatively with aerobic capacity (r=-0.408, r=-0.308, and r=-0.207, respectively). We observed similar correlations with OS markers (thiobarbituric acid and carbonyls; r=-0.290 and r=0.379, respectively), and these markers also significantly correlated with the aerobic capacity. CONCLUSIONS: Adults with bronchiectasis presented an increased systemic inflammatory response that correlated negatively with physical capacity.

Exercise-induced desaturation in subjects with non-cystic fibrosis bronchiectasis: laboratory-based tests versus field-based exercise tests.

OBJECTIVE: To investigate the validity of field walking tests to identify exercise-induced hypoxemia and to compare cardiorespiratory responses and perceived effort between laboratory-based and field-based exercise tests in subjects with bronchiectasis. METHODS: This was a cross-sectional study involving 72 non-oxygen-dependent participants (28 men; mean age = 48.3 ± 14.5 years; and mean FEV1 = 54.1 ± 23.4% of the predicted value). The participants underwent cardiopulmonary exercise testing (CPET) on a treadmill and constant work-rate exercise testing (CWRET) on the same day (1 h apart). In another visit, they underwent incremental shuttle walk testing (ISWT) and endurance shuttle walk testing (ESWT; 1 h apart). Desaturation was defined as a reduction in SpO2 ≥ 4% from rest to peak exercise. RESULTS: CPET results were compared with ISWT results, as were CWRET results with ESWT results. There was no difference in the magnitude of desaturation between CPET and ISWT (-7.7 ± 6.3% vs. -6.6 ± 5.6%; p = 0.10) and between CWRET and ESWT (-6.8 ± 5.8% vs. -7.2 ± 6.3%; p = 0.50). The incremental tests showed an agreement in the magnitude of desaturation in the desaturation and no desaturation groups (42 and 14 participants, respectively; p < 0.01), as did the endurance tests (39 and 16 participants; p < 0.01). The magnitude of desaturation was similar among the participants who did or did not reach at least 85% of the maximum predicted HR. CONCLUSIONS: Field exercise tests showed good precision to detect desaturation. Field tests might be an alternative to laboratory tests when the clinical question is to investigate exercise-induced desaturation in subjects with bronchiectasis.

Improving Airways Patency and Ventilation Through Optimal Positive Pressure Identified by Noninvasive Mechanical Ventilation Titration in Mounier-Kuhn Syndrome: Protocol for an Interventional, Open-Label, Single-Arm Clinical Trial.

BACKGROUND: Mounier-Kuhn syndrome or congenital tracheobronchomegaly is a rare disease characterized by dilation of the trachea and the main bronchi within the thoracic cavity. The predominant signs and symptoms of the disease include coughing, purulent and abundant expectoration, dyspnea, snoring, wheezing, and recurrent respiratory infection. Symptoms of the disease in some patients are believed to be pathological manifestations arising due to resident tracheobronchomalacia. Although treatment options used for the management of this disease include inhaled bronchodilators, corticosteroids, and hypertonic solution, there is no consensus on the treatment. The use of continuous positive airway pressure (CPAP) has been reported as a potential therapeutic option for tracheobronchomalacia, but no prospective studies have demonstrated its efficacy in this condition. OBJECTIVE: The purpose of this is to identify the presence of tracheobronchomalacia and an optimal CPAP pressure that reduces the tracheobronchial collapse in patients with Mounier-Kuhn syndrome and to analyze the repercussion in pulmonary ventilation. In parallel, we aim to evaluate the prevalence of obstructive sleep apnea/hypopnea syndrome. METHODS: This interventional, open-label, single-arm clinical trial will enroll patients who are diagnosed Mounier-Kuhn syndrome. Patient evaluation will be conducted in an outpatient clinic and involve 3 visits. Visit 1 will involve the collection and registration of social demographic, clinical, and functional data. Visit 2 will entail polysomnography, bronchoscopy for the evaluation of tracheobronchomalacia, titration of the optimal pressure that reduces the degree of collapse of the airway, and electrical impedance tomography. In visit 3, patients exhibiting a reduction in collapse areas will be requested to undergo chest computed tomography during inspiration and forced expiration with and without positive pressure (titrated to determine optimal CPAP pressure). RESULTS: This protocol is a doctorate project. The project was submitted to the institutional review board on January 24, 2017, and approval was granted on February 2, 2017 (Brazilian Research database number CAAE 64001317.4.000.0068). Patient evaluations started in April 2018. Planned recruitment is based on volunteers' availability and clinical stability, and interventions will be conducted at least once a month to finish the project at the end of 2020. A preliminary analysis of each case will be performed after each intervention, but detailed results are expected to be reported in the first quarter of 2021. CONCLUSIONS: There is no consensus on the best treatment options for managing Mounier-Kuhn syndrome. The use of positive pressure could maintain patency of the collapsed airways, functioning as a "pneumatic stent" to reduce the degree of airflow obstruction. This, in turn, could promote mobilization of thoracic secretion and improve pulmonary ventilation. TRIAL REGISTRATION: ClinicalTrails.gov NCT03101059; https://clinicaltrials.gov/ct2/show/NCT03101059. INTERNATIONAL REGISTERED REPORT IDENTIFIER (IRRID): DERR1-10.2196/14786.

'Go for it, dream big, work hard and persist': A message to the next generation of CF leaders in recognition of International Women's Day 2020.

The focus for International Women's Day 2020 is gender equity:'We can actively choose to challenge stereotypes, fight bias, broaden perceptions, improve situations and celebrate women's achievements. Collectively, each one of us can help create a gender equal world.' We have come together as an international group of women holding senior positions within CF to raise awareness. There is growing recognition of gender imbalance within our sector in senior leadership, grant and publication success. Several institutions, such as National Institutes of Health, have missions to tackle this. The issues raised by our panellists were wide-ranging: decisions around starting a family, impact on career progression; experiences of bias in appointments or promotions; selfbelief. We hope that raising these issues will encourage future leaders in CF to step up, to build teams based on fairness, equity and diversity, and to catalyse steps towards this goal in their institutions and society more widely.

Physiological Responses During Field Walking Tests in Adults with Bronchiectasis.

BACKGROUND: Field walking tests are commonly used in patients with chronic pulmonary diseases for assessment of functional capacity. However, the physiological demands and magnitude of desaturation on 6-min walk test (6MWT), incremental shuttle walk test (ISWT), and endurance shuttle walk test (ESWT) have not been investigated in patients with bronchiectasis. The objective of this study was to compare the physiological responses and the magnitude of desaturation of subjects with bronchiectasis when performing the 6MWT, ISWT, and ESWT. METHODS: Thirty-two subjects underwent the 6MWT, ISWT, and ESWT on 3 different days. Pulmonary gas exchange, heart rate, and [Formula: see text] were measured in all tests. RESULTS: There were no differences in the peak rate of oxygen uptake, ventilation, dyspnea, and leg fatigue between the tests. Equivalent cardiac demand (ie, heart rate at peak) was observed with the 6MWT (137 ± 21 beats/min) and the ESWT (142 ± 21 beats/min), but this was lower in the ISWT (135 ± 19 beats/min) compared to ESWT (P < .05). Most subjects achieved a vigorous exercise intensity (heart rate of 70-90% of predicted) in all tests. There was no difference in desaturation among the tests (6MWT: -6.8 ± 6.6%, ISWT: -6.1 ± 6.0%, and ESWT: -7.0 ± 5.4%). CONCLUSIONS: The 6MWT, ISWT, and ESWT induced similar physiological responses at the peak of exercise, eliciting a vigorous exercise intensity. The magnitude of desaturation was similar across tests. This means these tests can be used interchangeably for evaluation of exercise-induced desaturation.

Maternal and Perinatal Outcomes in Pregnant Women with Cystic Fibrosis.

OBJECTIVES: To assess the perinatal and maternal outcomes of pregnant women with cystic fibrosis (CF) and severe lung impairment. METHODS: This was a series of cases aiming to review the maternal and fetal outcomes in cases of singleton pregnant women with a diagnosis of CF. We have included all of the cases of singleton pregnancy in patients with CF who were followed-up at the obstetrics department of the Medical School of the Universidade de São Paulo, between 2003 and 2016. The exclusion criteria were the unattainability of the medical records of the patient, and delivery at other institutions. A forced expiratory volume in 1 second < 50% was considered as severe lung impairment. We have also analyzed data regarding maternal hospitalization and respiratory exacerbations (REs). RESULTS: Pregnant women with CF and severe lung impairment did not present an association with spontaneous prematurity, fetal growth restriction or fetal demise. All of the cases involved multiple RE episodes requiring antibiotic therapy. The median (range) of events per patient was of 4 (2-4) events. CONCLUSION: Cystic fibrosis patients with severe lung impairment may achieve successful term pregnancies. However, pregnancies of women with CF are frequently complicated by REs, and this population may require hospital admission during the course of the pregnancy. Cystic fibrosis patients should be followed by a specialized team with experience in treating respiratory diseases.

OBJETIVO: Avaliar os desfechos maternos e perinatais de gestações em mulheres portadoras de fibrose cística (FC) e disfunção pulmonar grave. MéTODOS: Série de casos visando a avaliação dos desfechos maternos e perinatais em gestações únicas de mulheres com diagnóstico de FC. Foram incluídos todos os casos de gestações únicas de pacientes com FC que tiveram acompanhamento no departamento de obstetrícia da Faculdade de Medicina da Universidade de São Paulo, no período de 2003 a 2016. Os critérios de exclusão foram não disponibilidade do prontuário da paciente e parto em outro serviço. Disfunção pulmonar grave foi definida como presença de volume expiratório forçado em 1 segundo < 50%. Foram analisados também os dados referentes a exacerbações respiratórias e internações maternas. RESULTADOS: Gestação em mulheres portadoras de FC com disfunção pulmonar grave não se associaram com prematuridade espontânea, restrição de crescimento fetal ou óbito fetal. Todos os casos apresentaram múltiplos episódios de exacerbações respiratórias necessitando de antibioticoterapia. A mediana de eventos por pacientes (intervalo) foi de 4 (2­4) eventos. CONCLUSãO: Mulheres portadoras de FC com disfunção pulmonar grave podem evoluir com gestações de termo bem sucedidas. Entretanto, gestações nestas pacientes são frequentemente complicadas por exacerbações respiratórias, necessitando de internação. Gestantes portadoras de FC devem ser acompanhadas por uma equipe especializada com experiência no manejo de doenças respiratórias.

Prognostic Value of Frequent Exacerbations in Bronchiectasis: The Relationship With Disease Severity.

INTRODUCTION: Bronchiectasis is a very heterogeneous disease but some homogeneous groups with similar clinical characteristics and prognosis have been identified. Exacerbations have been shown to have a negative impact on the natural history of bronchiectasis. The objective of this study was to identify the definition and characteristics of the "frequent exacerbator patient" with the best prognostic value and its relationship with the severity of bronchiectasis. METHODS: A historical cohort of 651 patients diagnosed with bronchiectasis was included. They had all received 5 years of follow-up since their radiological diagnosis. Exacerbation was defined as a worsening of the symptoms derived from bronchiectasis that required antibiotic treatment. The main outcome was all-cause mortality at the end of follow-up. RESULTS: The mean age was 48.2 (16) years (32.9% males). 39.8% had chronic infection by Pseudomonas aeruginosa. Mean BSI, FACED, and E-FACED were 7 (4.12), 2.36 (1.68), and 2.89 (2.03), respectively. There were 95 deaths during follow-up. The definition of the "frequent exacerbator patient" that presented the greatest predictive power for mortality was based on at least two exacerbations/year or one hospitalization/year (23.3% of patients; AUC-ROC: 0.75 [95% CI: 0.69-0.81]). Its predictive power was independent of the patient's initial severity. The clinical characteristics of the frequent exacerbator patient according to this definition varied according to the initial severity of bronchiectasis, presence of systemic inflammation, and treatment. CONCLUSIONS: The combination of two exacerbations or one hospitalization per year is the definition of frequent exacerbator patient that has the best predictive value of mortality independent of the initial severity of bronchiectasis.

Severe pulmonary disease in an adult primary ciliary dyskinesia population in Brazil.

Primary Ciliary Dyskinesia (PCD) is underdiagnosed in Brazil. We enrolled patients from an adult service of Bronchiectasis over a two-year period in a cross-sectional study. The inclusion criteria were laterality disorders (LD), cough with recurrent infections and the exclusion of other causes of bronchiectasis. Patients underwent at least two of the following tests: nasal nitric oxide, ciliary movement and analysis of ciliary immunofluorescence, and genetic tests (31 PCD genes + CFTR gene). The clinical characterization included the PICADAR and bronchiectasis scores, pulmonary function, chronic Pseudomonas aeruginosa (cPA) colonization, exhaled breath condensate (EBC) and mucus rheology (MR). Forty-nine of the 500 patients were diagnosed with definite (42/49), probable (5/49), and clinical (2/49) PCD. Twenty-four patients (24/47) presented bi-allelic pathogenic variants in a total of 31 screened PCD genes. A PICADAR score > 5 was found in 37/49 patients, consanguinity in 27/49, LD in 28/49, and eight PCD sibling groups. FACED diagnosed 23/49 patients with moderate or severe bronchiectasis; FEV1 ≤ 50% in 25/49 patients, eight patients had undergone lung transplantation, four had been lobectomized and cPA+ was determined in 20/49. The EBC and MR were altered in all patients. This adult PCD population was characterized by consanguinity, severe lung impairment, genetic variability, altered EBC and MR.

Brazilian consensus on non-cystic fibrosis bronchiectasis.

Bronchiectasis is a condition that has been increasingly diagnosed by chest HRCT. In the literature, bronchiectasis is divided into bronchiectasis secondary to cystic fibrosis and bronchiectasis not associated with cystic fibrosis, which is termed non-cystic fibrosis bronchiectasis. Many causes can lead to the development of bronchiectasis, and patients usually have chronic airway symptoms, recurrent infections, and CT abnormalities consistent with the condition. The first international guideline on the diagnosis and treatment of non-cystic fibrosis bronchiectasis was published in 2010. In Brazil, this is the first review document aimed at systematizing the knowledge that has been accumulated on the subject to date. Because there is insufficient evidence on which to base recommendations for various treatment topics, here the decision was made to prepare an expert consensus document. The Brazilian Thoracic Association Committee on Respiratory Infections summoned 10 pulmonologists with expertise in bronchiectasis in Brazil to conduct a critical assessment of the available scientific evidence and international guidelines, as well as to identify aspects that are relevant to the understanding of the heterogeneity of bronchiectasis and to its diagnostic and therapeutic management. Five broad topics were established (pathophysiology, diagnosis, monitoring of stable patients, treatment of stable patients, and management of exacerbations). After this subdivision, the topics were distributed among the authors, who conducted a nonsystematic review of the literature, giving priority to major publications in the specific areas, including original articles, review articles, and systematic reviews. The authors reviewed and commented on all topics, producing a single final document that was approved by consensus.

Reduced Physical Activity With Bronchiectasis.

BACKGROUND: Bronchiectasis leads to reduced functional capacity, which might have implications for physical activity. The impact of dyspnea and long-term oxygen therapy on physical activity has never been investigated in subjects with bronchiectasis. Based on these findings, specific strategies could be applied to allow individuals to be more active in their daily life. In this study we aimed to evaluate physical activity, the impact of dyspnea and long-term oxygen therapy on physical activity, and the determinants of physical activity in subjects with bronchiectasis. METHODS: We performed a cross-sectional study in 139 subjects with bronchiectasis (age 45 ± 13 y, FVC 70 ± 22% of predicted, FEV1 54 ± 25% of predicted) and 49 healthy subjects as controls. Physical activity was assessed using steps per day (measured with a pedometer), spirometry, incremental shuttle walking test (ISWT), and dyspnea. RESULTS: Reduced physical activity was observed in subjects with bronchiectasis, who recorded a median (interquartile range) of 8,007 (5,131-10,432) steps/d compared with controls, who recorded 10,994 (8,551-14,078) steps/d (P < .001). Significant correlations were observed between physical activity and FVC (r = 0.43), FEV1 (r = 0.36), ISWT (r = 0.37), and dyspnea (r = -0.48). Determinants for reduced physical activity included pulmonary function (R2 = 0.150), dyspnea (R2 = 0.075), ISWT (R2 = 0.044), and long-term oxygen therapy (R2 = 0.038); these factors explained 32% of the physical activity. CONCLUSION: Subjects with bronchiectasis exhibited reduced physical activity compared with healthy peers. Dyspnea has a negative impact on physical activity. Independent factors associated with physical activity included pulmonary function, dyspnea, functional capacity, and long-term oxygen therapy. These findings will guide strategies to enhance daily physical activity and to encourage subjects with bronchiectasis to be more active.