The objective of this study was to assess the efficacy of exercise training by means of a rehabilitative walking protocol. Twenty patients with COPD staged as III/IV according to the Global Initiative for Chronic Obstructive Lung Disease were included. Patients were evaluated at baseline and at the end of the exercise program regarding oxygen desaturation, sensation of dyspnea/fatigue, quality of life and six-minute walk distance. The comparison between the six-minute walk distance prior to and after training proved the efficacy of the protocol, which also resulted in a decrease in oxygen desaturation and in the sensation of dyspnea. This protocol can be easily put into practice.

Keywords: Activities of daily living; Pulmonary disease, chronic obstructive; Rehabilitation; Exercise therapy; Exercise test.

Objective: To evaluate weight changes after 12 months of biochemically confirmed smoking abstinence, comparing patients who lost weight or maintained their baseline weight with those who gained weight. Methods: This was a real-world prospective cohort study conducted at the Outpatient Smoking Cessation Clinic of São Lucas Hospital, in the city of Porto Alegre, Brazil, between 2010 and 2016. The patients evaluated received intensive smoking cessation counseling, focused especially on weight issues, together with pharmacotherapy, and were followed for 12 months. The baseline and final weights were measured. Continuous abstinence was confirmed by determining the concentration of exhaled carbon monoxide (eCO). Results: Of a total of 348 patients evaluated, 161 (46.2%) achieved continuous abstinence (eCO < 10 ppm) over the 12-month follow-up period. Of those 161 patients, 104 (64.6%) maintained their initial weight or had a weight change of no more than 5% in relation to their baseline weight, whereas the remaining 57 (35.4%) had a weight gain of more than 5%, 18 of those patients showing a > 10% increase over their baseline weight. The number needed to harm (i.e., the number of patients required in order to detect one patient with a weight increase) was calculated to be 3.6 (95% CI: 2.8-5.4). Conclusions: Weight gain is not necessarily associated with smoking cessation, and smokers who are motivated to quit should be informed of that fact. This information could also be useful for addressing smokers who are still undecided because of possibility of weight gain.

Keywords: Weight loss; Smoking cessation; Tobacco smoking; Treatment outcome.

Objective: To analyze the characteristics of cystic fibrosis (CF) care centers (CFCCs) in Brazil. Methods: A questionnaire was sent to the coordinators of all 51 registered CFCCs between May and September of 2021. Results: The response rate was 100%. Southeastern Brazil is the region where most of the CFCCs in the country are located (21 centers; 41%), followed by the southern and northeastern regions (11 centers each; 21.5%), the central-western region (6; 12%), and the northern region (2; 4%). A total of 4,371 patients with CF were cared for in Brazil during the study period, ranging from 7 to 240 patients per center (mean, 86 patients/center; median, 75 patients/center); 2,197 patients (50%) were cared for in centers in the southeastern region of the country, particularly in the state of São Paulo (33%), the remaining patients being treated in southern Brazil (1,014 patients, 23%), northeastern Brazil (665 patients, 15%), central-western Brazil (354 patients, 8%), and northern Brazil (141 patients, 4%). Overall, 47 centers (92%) reported having an incomplete multidisciplinary team; 4 (8%) lacked essential team members; 6 (12%) lacked a physical therapist; 5 (10%) lacked a dietitian; 17 (33%) lacked outpatient nursing care; 13 (25%) lacked outpatient social work services; 14 (27%) lacked a psychologist; and 32 (63%) lacked a clinical pharmacist. Seven CFCCs (14%) in the northern and northeastern regions of Brazil reported that the quality of newborn screening for CF was poor. All centers reported having difficulties in accessing CF medications. Conclusions: Brazilian CFCCs experience multiple problems, including inadequate staffing, infrastructure, testing, and medication supply. There is an urgent need to regulate the implementation of CF referral centers and an appropriate network structure for the diagnosis and follow-up of CF patients using optimal treatment recommendations.

Keywords: Cystic fibrosis; Neonatal screening; Quality of life; Genetic diseases, inborn; Lung diseases.

Objective: To evaluate the effectiveness of treatment with elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) and to characterize its safety profile in cystic fibrosis (CF) patients in a real-world clinical setting. Methods: This was a prospective observational study carried out in a CF referral center in Portugal involving adult CF patients who started treatment with ELX/TEZ/IVA. Clinical characteristics of the patients were collected, and effectiveness and safety data were evaluated. Results: Of the 56 patients followed in the center at the time of the study, 28 were eligible for ELX/TEZ/IVA treatment in accordance with the Portuguese National Authority for Medicines and Health Products at the time of the study. Of these, 24 met the follow-up time requirement to be included in the clinical effectiveness analysis. The mean follow-up time was 167.3 ± 96.4 days. Adverse events were generally mild and self-limited. Significant improvements in lung function, BMI, sweat chloride concentration, and number of pulmonary exacerbations were observed. No significant differences in outcomes between F508del homozygous and heterozygous patients were found. The effectiveness of this new CFTR modulator combination also applied to patients with advanced lung disease. Conclusions: Treatment with ELX/TEZ/IVA showed effective improvement in real-world clinical practice, namely in lung function, BMI, sweat chloride concentration, and number of pulmonary exacerbations, with no safety concerns.

Keywords: Cystic fibrosis; Cystic fibrosis transmembrane conductance regulator; Membrane transport modulators; Treatment outcome.

Objective: To describe COPD pharmacological treatment patterns in the state of Bahia, Brazil, and to evaluate the extent to which these patterns conform to clinical guidelines for the management of COPD. Methods: This was a cross-sectional study of 441 patients referred from the Public Health Care Network of the state of Bahia to a public referral outpatient clinic of a COPD management program of the Brazilian Unified Health Care System. Individuals with a spirometry-confirmed diagnosis of moderate to very severe COPD were included in the study. Patients were evaluated as to whether they had used any COPD medications in the last seven days. The appropriateness or inappropriateness (undertreatment or overtreatment) of the patient's pharmacological treatment was evaluated by comparing the patient's current treatment with that recommended by national and international guidelines. Results: A total of 383 individuals were included in the analysis. Approximately half of the patients (49.1%) used long-acting bronchodilators. These patients were older and had had the disease longer. Of the sample as a whole, 63.7% and 83.0% did not receive pharmacological treatment in accordance with international and national recommendations, respectively. Inappropriateness due to undertreatment was indentified in more than half of the patients. Conclusions: Long-acting bronchodilators are frequently underused in individuals with moderate to very severe COPD within the Brazilian Unified Health Care System in the state of Bahia. Most patients in our sample were treated inappropriately, and undertreatment predominated. Strategies to improve access to long-acting bronchodilators and the quality of COPD pharmacological management are required.

Keywords: Pulmonary disease, chronic obstructive/therapy; Drug therapy; Clinical protocols