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.

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).

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.

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.

Characteristics and outcomes of patients with COVID-19 admitted to the ICU in a university hospital in São Paulo, Brazil - study protocol.

Objectives: We designed a cohort study to describe characteristics and outcomes of patients with coronavirus disease (COVID-19) admitted to the intensive care unit (ICU) in the largest public hospital in Sao Paulo, Brazil, as Latin America becomes the epicenter of the pandemic.

Methods: This is the protocol for a study being conducted at an academic hospital in Brazil with 300 adult ICU beds dedicated to COVID-19 patients. We will include adult patients admitted to the ICU with suspected or confirmed COVID-19 during the study period.

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.

'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.

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.

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.

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.

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.

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.

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.

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.

The annual prognostic ability of FACED and E-FACED scores to predict mortality in patients with bronchiectasis.

http://ow.ly/albl30i11bv.

Determinants of Peripheral Muscle Strength and Activity in Daily Life in People With Bronchiectasis.

Background: Bronchiectasis is characterized by a progressive structural lung damage, recurrent infections and chronic inflammation which compromise the exertion tolerance, and may have an impact on skeletal muscle function and physical function.

Objective: The purpose of this study was to compare peripheral muscle strength, exercise capacity, and physical activity in daily life between participants with bronchiectasis and controls and to investigate the determinants of the peripheral muscle strength and physical activity in daily life in bronchiectasis.

Design: This study used a cross-sectional design.

Does home-based pulmonary rehabilitation improve functional capacity, peripheral muscle strength and quality of life in patients with bronchiectasis compared to standard care?

Background: Home-based pulmonary rehabilitation is a promising intervention that may help patients to overcome the barriers to undergoing pulmonary rehabilitation. However, home-based pulmonary rehabilitation has not yet been investigated in patients with bronchiectasis.

Objectives: To investigate the effects of home-based pulmonary rehabilitation in patients with bronchiectasis.

Brazilian guidelines for the diagnosis and treatment of cystic fibrosis.

Cystic fibrosis (CF) is an autosomal recessive genetic disorder characterized by dysfunction of the CFTR gene. It is a multisystem disease that most often affects White individuals. In recent decades, various advances in the diagnosis and treatment of CF have drastically changed the scenario, resulting in a significant increase in survival and quality of life.