Objective: To analyze and compare the transport properties of respiratory secretions, classified by selected parameters, in individuals with bronchiectasis unrelated to cystic fibrosis. Methods: We collected mucus samples from 35 individuals with bronchiectasis unrelated to cystic fibrosis. The samples were first classified by their surface properties (adhesive or nonadhesive), as well as by their aspect (mucoid or purulent). We then tested the samples regarding relative transport velocity (RTV), displacement in a simulated cough machine (SCM), and contact angle (CA). For the proposed comparisons, we used ANOVA models, with a level of significance set at 5%. Results: In comparison with nonadhesive samples, adhesive samples showed significantly less displacement in the SCM, as well as a significantly higher CA (6.52 ± 1.88 cm vs. 8.93 ± 2.81 cm and 27.08 ± 6.13° vs. 22.53 ± 5.92°, respectively; p < 0.05 for both). The same was true in the comparison between purulent and mucoid samples (7.57 ± 0.22 cm vs. 9.04 ± 2.48 cm and 25.61 ± 6.12° vs. 21.71 ± 5.89°; p < 0.05 for both). There were no significant differences in RTV among the groups of samples, although the values were low regardless of the surface properties (adhesive: 0.81 ± 0.20; nonadhesive: 0.68 ± 0.24) or the aspect (purulent: 0.74 ± 0.22; mucoid: 0.82 ± 0.22). Conclusions: The respiratory secretions of patients with bronchiectasis showed decreased mucociliary transport. Increased adhesiveness and purulence cause the worsening of transport properties, as demonstrated by the lesser displacement in the SCM and the higher CA.

Keywords: Bronchiectasis; Mucus; Mucociliary clearance; Adhesiveness.

Multiple respiratory abnormalities can be found in anxiety disorders, especially in panic disorder (PD). Individuals with PD experience unexpected panic attacks, characterized by anxiety and fear, resulting in a number of autonomic and respiratory symptoms. Respiratory stimulation is a common event during panic attacks. The respiratory abnormality most often reported in PD patients is increased CO2 sensitivity, which has given rise to the hypothesis of fundamental abnormalities in the physiological mechanisms that control breathing in PD. There is evidence that PD patients with dominant respiratory symptoms are more sensitive to respiratory tests than are those who do not manifest such symptoms, and that the former group constitutes a distinct subtype. Patients with PD tend to hyperventilate and to panic in response to respiratory stimulants such as CO2, triggering the activation of a hypersensitive fear network. Although respiratory physiology seems to remain normal in these subjects, recent evidence supports the idea that they present subclinical abnormalities in respiration and in other functions related to body homeostasis. The fear network, composed of the hippocampus, the medial prefrontal cortex, the amygdala and its brain stem projections, might be oversensitive in PD patients. This theory might explain why medication and cognitive-behavioral therapy are both clearly effective. Our aim was to review the relationship between respiration and PD, addressing the respiratory subtype of PD and the hyperventilation syndrome, with a focus on respiratory challenge tests, as well as on the current mechanistic concepts and the pharmacological implications of this relationship.

Keywords: Panic disorder; Anxiety; Respiration; Hyperventilation; Carbon dioxide.

Objective: To evaluate the prevalence of alpha-1 antitrypsin (AAT) variants through SERPINA1 genotyping in patients with non-cystic fibrosis bronchiectasis, and assess their clinical, functional and radiological characteristics. AAT deficiency is underdiagnosed, and an etiology to be considered when evaluating bronchiectasis. Methods: A cross-sectional study was conducted at an outpatient clinic focused on bronchiectasis in a tertiary hospital. Data from patients followed between 2005 and 2023 were collected. Genotyping for AAT was performed. Demographic, clinical, pulmonary function tests, serum AAT levels and chest CT data were analyzed. Results: A total of 136 patients were included, predominantly female (72.1%), with a median age of 56.6 years. The prevalence of SERPINA1 gene mutations was 25.7% (n=35). Among the detected variant genotypes were Pi*MS (15.4%), Pi*MZ (5,1%), Pi*SS (1,5%), Pi*ZZ (1,5%), Pi*MI (0,7%), Pi*SZ (0,7%) and Pi*ZMMalton (0,7%). When comparing patients with and without SERPINA1 mutations, significant differences were observed in AAT serum levels, emphysema type (panlobular) and distribution (diffuse and lower-lobe predominant). No other clinical, microbiological, functional or radiological differences were found, including emphysema presence or absence. Notably, 16 (45.7%) of individuals carrying SERPINA1 mutations exhibited normal serum AAT levels. Conclusions: AAT variants are not uncommon among patients with bronchiectasis. Presence of panlobular, diffuse or lower-lobe predominant emphysema should prompt AATD diagnostic consideration. However, the absence of emphysema does not exclude the diagnosis. Moreover, SERPINA1 variants may occur along with normal AAT serum levels. Clinicians should consider genotyping in patients with normal AAT levels, particularly when bronchiectasis remains unexplained.

Keywords: Alpha 1-antitrypsin deficiency; Bronchiectasis; Genotype; Mutation; Alleles

Objective: To determine the occurrence of cardiorespiratory alterations and to identify adverse events during the intrahospital transport of patients on invasive ventilation. Methods: A prospective observational non-randomized study was conducted at two tertiary hospitals between April of 2005 and December of 2006. We included patients on invasive ventilation who required intrahospital transport during the study period. Exclusion criteria were as follows: being under suspicion of brain death; being submitted to alternate periods of mechanical ventilation/nebulization via a T-piece; and being transported to the operating room. Prior to and after transport, we evaluated blood gas analysis results, vital signs, use of medications by means of a continuous infusion pump, parameters regarding the mechanical ventilator, duration of transport, transport distance and number of professionals involved. Results: We included 48 patients in a total of 58 intrahospital transports. Relevant cardiorespiratory alterations were identified in 39 transports, totaling 86 episodes, as well as 16 adverse events related to equipment or personnel failure, such as problems related to batteries and to miscommunication. Conclusions: During the intrahospital transport of patients on invasive ventilation, cardiorespiratory alterations were common (67.2%), and adverse events occurred in 75.7% of the transports.

Keywords: Patient transfer; Intensive care; Respiration, artificial; Ventilators, mechanical.

Objective: To evaluate the effects of azathioprine on the mucociliary system in a model of bronchial section and anastomosis in rats. Methods: Thirty-six male Wistar-Furth rats were submitted to left bronchial section and anastomosis and divided into two groups to receive either saline solution or azathioprine. After 7, 15 and 30 days of treatment, six animals from each group were killed, after which in situ mucociliary transport velocity, in vitro mucus transportability, and contact angle of mucus in the right (intact) and left (sectioned) bronchi were measured. Results: In situ mucociliary transport velocity was significantly lower in the sectioned bronchi than in the intact bronchi (p < 0.001). In situ mucociliary transport velocity was lower in the intact bronchi of the animals treated with azathioprine for 7 days (p < 0.05), and those bronchi presented full recovery after 30 days of treatment. The contact angle was higher in the mucus samples collected from the sectioned bronchi of the animals treated with saline solution for 30 days (p < 0.001), which is in accordance with the decreased in vitro mucus transportability observed in the same animals (p < 0.001). Conclusions: We conclude that, in the sectioned bronchi of rats, treatment with azathioprine causes only transitory impairment of mucociliary transport, whereas administration of saline solution impairs mucociliary transport for up to 30 days. In addition, azathioprine protects against alterations in mucus surface properties.

Keywords: Mucociliary clearance; Anastomosis, surgical; Immunosuppression; Lung.

Objective: To characterize a population of patients with bronchiectasis, correlating clinical, radiological, and functional aspects with the severity of dyspnea. Methods: This was a cross-sectional study involving adult patients with HRCT-confirmed bronchiectasis, categorized according to the severity of dyspnea (as being mildly or severely symptomatic, on the basis of the modified Medical Research Council scale). We correlated the severity of dyspnea with clinical parameters, functional parameters (spirometry values, lung volumes, and DLCO), and CT parameters. Results: We evaluated 114 patients, 47 (41%) of whom were men. The median age (interquartile range) was 42 years (30-55 years). The most common form was idiopathic bronchiectasis. Of the 114 patients, 20 (17.5%) were colonized with Pseudomonas aeruginosa and 59 (51.8%) were under continuous treatment with macrolides. When we applied the Exacerbation in the previous year, FEV1, Age, Colonization, Extension, and Dyspnea score, the severity of dyspnea was categorized as moderate in 54 patients (47.4%), whereas it was categorized as mild in 50 (43.9%) when we applied the Bronchiectasis Severity Index. The most common lung function pattern was one of obstruction, seen in 95 patients (83.3%), and air trapping was seen in 77 patients (68.7%). The prevalence of an obstructive pattern on spirometry was higher among the patients with dyspnea that was more severe, and most functional parameters showed reasonable accuracy in discriminating between levels of dyspnea severity. Conclusions: Patients with bronchiectasis and dyspnea that was more severe had greater functional impairment. The measurement of lung volumes complemented the spirometry data. Because bronchiectasis is a complex, heterogeneous condition, a single variable does not seem to be sufficient to provide an overall characterization of the clinical condition.

Keywords: Bronchiectasis; Dyspnea; Respiratory function tests; Multidetector computed tomography; Plethysmography.

Objective: To identify microorganisms in sputum samples of patients with stable non-cystic fibrosis bronchiectasis and to determine risk factors related to the isolation of Pseudomonas aeruginosa (PA) in those patients. Methods: Consecutive patients were recruited from a tertiary hospital outpatient clinic in the city of Fortaleza, Brazil. The patients were submitted to spirometry, six-minute walk test, HRCT, and sputum collection. Data on serum fibrinogen levels, disease severity, sputum color, and history of azithromycin treatment were collected. Results: The study included 112 patients, and females predominated (68%). The mean age was 51.6 ± 17.4 years. Most patients presented with mild-to-moderate disease (83%). The mean six-minute walk distance was 468.8 ± 87.9 m. Mean FEV1 and FVC, in % of predicted values, were 60.4 ± 21.8% and 69.9 ± 18.5%, respectively. The mean serum fibrinogen level was 396.1 ± 76.3 mg/dL. PA was isolated in 47 patients, other potentially pathogenic microorganisms (PPMs) were isolated in 31 patients, and non-PPMs were isolated in 34 patients. Purulent sputum was identified in 77 patients (68%). The patients with PA, when compared with those without it, presented with more severe disease, higher serum fibrinogen levels, and lower FVC%. In addition, purulent sputum and long-term azithromycin treatment were more common in those with PA. The multivariate regression analysis showed that the independent factors associated with PA were serum fibrinogen level > 400 mg/dL (OR = 3.0; 95% CI: 1.1-7.7) and purulent sputum (OR = 4.3; 95% CI: 1.6-11.3). Conclusions: In our sample, the prevalence of PA in sputum was 42%. Sputum color and inflammatory markers were able to predict the isolation of PA, emphasizing the importance of routine sputum monitoring.

Keywords: Bronchiectasis; Pseudomonas aeruginosa; Sputum/microbiology.

Objective: To identify reference values for handgrip strength through a literature search and compare the agreement of reference values from Brazil with others for handgrip strength in a sample of COPD patients in Brazil, as well as to determine which set of reference values is more discriminative regarding differences in clinical characteristics between individuals with low handgrip strength and normal handgrip strength. Methods: To identify reference values for handgrip strength, a literature search was performed; a retrospective cross-sectional analysis of baseline-only data from two unrelated studies was then performed. Individuals were evaluated for handgrip strength, peripheral muscle strength, respiratory muscle strength, pulmonary function, body composition, exercise capacity, dyspnea, and functional status. Results: Of the 45 studies that were initially selected, 9 met the criteria for inclusion in the analysis, which included 99 COPD patients in Brazil (52% of whom were male with GOLD stage II-IV COPD). The prevalence of low handgrip strength varied across studies (from 9% to 55%), the set of reference values for handgrip strength in a sample of individuals in Brazil having classified 9% of the study sample as having low handgrip strength. The level of agreement between the reference values for a sample of individuals in Brazil and the other sets of reference values varied from weak to excellent. The reference values for a sample of individuals in Brazil showed the highest number of significantly different characteristics between individuals with low and normal handgrip strength. Conclusions: The level of agreement between national and international sets of reference values for handgrip strength varied from weak to excellent in COPD patients in Brazil. Reference values for handgrip strength with higher discriminative capacity are not necessarily those that identify more individuals as having low handgrip strength.

Keywords: Pulmonary disease, chronic obstructive; Hand strength; Muscle weakness; Muscle strength; Reference values.

Nitric oxide (NO) is an endogenous vasoactive compound that contributes to pulmonary vascular homeostasis and is produced by three nitric oxide synthase (NOS) isoforms-neuronal NOS (nNOS); inducible NOS (iNOS); and endothelial NOS (eNOS)-all three of which are present in the lung. Studies using pharmacological inhibitors or knockout mice have shown that eNOS-derived NO plays an important role in modulating pulmonary vascular tone and attenuating pulmonary hypertension. However, studies focusing on the role of iNOS have shown that this isoform contributes to the pathophysiology of acute lung injury and acute respiratory distress syndrome. This review aimed at outlining the role played by NO in the control of pulmonary circulation, both under physiological and pathophysiological conditions. In addition, we review the evidence that the L-arginine-NO-cyclic guanosine monophosphate pathway is a major pharmacological target in the treatment of pulmonary vascular diseases.

Keywords: Nitric oxide; Arginine; Nitric oxide synthase; Cyclic GMP; Pulmonary circulation.

Background: Bronchiectasis is a frequently found disease in medical practice in Brazil leading to significant morbidity and decrease in quality of life of the affected individuals. Objectives: To study diagnostic and therapeutic aspects in a series of hospitalized patients with bronchiectasis in a department of pulmonary diseases. Methods: Signs, symptoms, microbiological and radiographic data, and therapeutic results were studied in 170 hospitalized patients between 1978 and 2001 - females 62.4%, males 37.6%, and aged from 12 to 88 years (mean age 36.8 yrs). Previous history of pneumonia in childhood was detected in 52.5% of the patients, tuberculosis in 19.8%; 8.8% had bronchial asthma, and 2 had Kartagener's syndrome. Results: The most common symptoms were cough (100.0%), expectoration (96.0%) and pulmonary rales (66.0%). The pulmonary lesions were unilateral in 46.5% of the cases. Pneumococcus, H. influenzae or mixed flora were found in 85.0% of the examined sputa. All 170 patients received antibiotics and postural drainage, and 88 of them (younger and with a higher functional reserve) were also submitted to pulmonary resections (82 unilateral and 6 bilateral). Two deaths occurred, and repetitive hospitalizations were more frequent among the clinically treated patients. The follow up showed that most of the surgically treated patients had significant symptoms improvement and rarely needed to be re-hospitalized. Conclusions: In the majority of the patients, lung resection surgery improved permanently the prolonged bronchopulmonary symptoms of patients with bronchiectasis, differently from the patients who received only clinical treatment.

Keywords: Bronchiectasis/diagnosis. Bronchiectasis/therapy. Bronchiectasis/surgery. Bronchiectasis/complications. Tomography X-ray computed/methods. Inpatients. Retrospective studies.

Objective: To determine the effectiveness of oscillating positive expiratory pressure (OPEP) using predetermined expiratory pressures on the viscosity and transportability of sputum in patients with bronchiectasis. Methods: The study involved 15 stable patients with bronchiectasis (7 males; mean age = 53 ± 16 years), submitted to two consecutive OPEP interventions, with a 24-h interval between the two, using positive expiratory pressures set at 15 cmH2O (P15) and 25 cmH2O (P25). The protocol consisted of a voluntary cough; another voluntary cough 20 min later, designated time zero (T0); a 10-min rest period; and two 10-min series (S1 and S2, using OPEP at P15 and P25 in both), with a 10-min interval between the two. The viscosity and transportability of sputum were evaluated by viscometry, relative transport velocity on frog palate, transport in a simulated cough machine and contact angle. Sputum samples were collected at T0, after S1 and after S2. Specific statistical tests were performed depending on the type of data distribution. Results: In comparison with the values obtained at T0, sputum viscosity decreased significantly after S1 at P15 and after S2 at P25. There were no significant differences among all of the samples in terms of transportability. Conclusions: The fact that sputum viscosity decreased whether OPEP was performed at P15 or at P25 suggests that there is no need to generate high expiratory pressure to achieve the desired result.

Keywords: Viscosity; Bronchiectasis; Physical therapy (specialty); Sputum.

Objective: To evaluate physical performance on the six-minute walk test (6MWT) in patients with non-cystic fibrosis bronchiectasis and to investigate its relationship with quality of life (QoL). To identify predictors of exercise performance, we also investigated whether six-minute walk distance (6MWD) is associated with clinical and spirometric findings. Methods: This was a cross-sectional study involving patients with non-cystic fibrosis bronchiectasis (age,  18 years), with at least one respiratory symptom for  2 years and an FEV1  70% of predicted. Patients underwent clinical evaluation, pulmonary function tests, the 6MWT, and QoL assessment with the Medical Outcomes Study 36-item Short-Form Health Survey (SF-36). Results: We included 70 patients (48 females). Mean age was 54.5 ± 17.7 years, and mean FEV1 was 44.9 ± 14.5% of predicted. The patients were divided into two groups: 6MWD-low (6MWD below the predicted lower limit; n = 23); and 6MWD-norm (normal 6MWD; n = 47). The following variables were significantly lower in the 6MWD-low group than in the 6MWD-norm group: age; age at diagnosis of bronchiectasis; proportion of former smokers; body mass index (BMI); FEV1% of predicted; and MEP% of predicted. There were no significant differences in the SF-36 scores between the groups. In the logistic regression model, lower age and lower BMI were significantly associated with lower 6MWD. Conclusions: In this sample, there was a high proportion of patients who had a lower than expected 6MWD. Although 6MWD was not related to QoL, it was associated with age and BMI.

Keywords: Bronchiectasis; Quality of life; Respiratory function tests; Exercise tolerance.

Objective: To evaluate the magnitude of diurnal changes in the parameters of pulmonary function and respiratory muscle strength/endurance in a sample of patients with COPD. Methods: A group of 7 patients underwent spirometry, together with determination of MIP and MEP, at two distinct times (between 8:00 and 8:30 a.m. and between 4:30 and 5:00 p.m.) on a single day. Between assessments, the patients remained at rest in the laboratory. Results: In accordance with the Global Initiative for Chronic Obstructive Pulmonary Disease staging system, COPD was classified as moderate, severe, and very severe in 1, 3, and 3 of the patients, respectively. From the first to the second assessment, there were significant decreases in FVC, FEV1, and MEP (of 13%, 15%, and 10%, respectively), as well as (less than significant) decreases in PEF, MIP, and maximal voluntary ventilation (of 9%, 3%, and 11%, respectively). Conclusions: In this sample of COPD patients, there were diurnal variations in the parameters of pulmonary function and respiratory muscle strength. The values of FEV1, FVC, and MEP were significantly lower in the afternoon than in the morning.

Keywords: Pulmonary disease, chronic obstructive; Respiratory function tests; Respiratory muscles.

Nonadherence to inhaled corticosteroid therapy is common and has a negative effect on clinical control, as well as increasing morbidity rates, mortality rates and health care costs. This review was conducted using direct searches, together with the following sources: Medline; HighWire; and the Latin American and Caribbean Health Sciences Literature database. Searches included articles published between 1992 and 2008. The following methods of assessing adherence, listed in ascending order by degree of objectivity, were identified: patient or family reports; clinical judgment; weighing/dispensing of medication, electronic medication monitoring; and (rarely) biochemical analysis. Adherence rates ranged from 30 to 70%. It is recognized that the degree of adherence determined by patient/family reports or by clinical judgment is exaggerated in comparison with that obtained using electronic medication monitors. Physicians should bear in mind that true adherence rates are lower than those reported by patients, and this should be considered in cases of poor clinical control. Weighing the spray quantifies the medication and infers adherence. However, there can be deliberate emptying of inhalers and medication sharing. Pharmacies provide the dates on which the medication was dispensed and refilled. This strategy is valid and should be used in Brazil. The use of electronic medication monitors, which provide the date and time of each triggering of the medication device, although costly, is the most accurate method of assessing adherence. The results obtained with such monitors demonstrate that adherence was lower than expected. Physicians should improve their knowledge on patient adherence and use accurate methods of assessing such adherence.

Keywords: Patient compliance; Asthma/therapy; Asthma/prevention & control.

Abstract Objective: To determine the prevalence of gastroesophageal reflux disease (GERD) and to evaluate the esophageal motor profile of patients with respiratory symptoms referred to a digestive motility referral center for esophageal function testing. Methods: The results of esophageal manometry and 24-h esophageal pH-metry were analyzed. The inclusion criterion was presenting respiratory symptoms, with or without accompanying digestive symptoms. Results: Of the 1,170 patients included in the study, 602 (51.5%) reported having digestive and respiratory symptoms (DRS group), and 568 (48.5%) reported having only respiratory symptoms (RS group). Asthma was diagnosed in 142 patients in the RS group (RS-A subgroup) and in 201 of those in the DRS group (DRS-A subgroup). Of the 346 cases of esophageal dysmotility, hypomotility was found in 175 (14.3% and 15.6% in the DRS and RS groups, respectively), and lower esophageal sphincter (LES) hypotonia was found in 411 (40.3% and 30.2%, respectively). Hypotonia correlated with GERD. Exposure of the distal esophagus to acid was markedly abnormal in the supine position. The prevalence of GERD in the sample as a whole, the RS-A/DRS-A subgroups and the RS-A subgroup alone was 39.8%, 44.0% and 35.2%, respectively. Conclusions: Hypotonic LES was the most common abnormality and correlated with GERD. Although GERD was more evident in the DRS group, approximately one third of the patients in the RS group also presented GERD (silent GERD). The findings suggest that GERD can be an extrapulmonary cause of chronic respiratory symptoms unresponsive to conventional therapy.

Keywords: Gastroesophageal reflux; Signs and symptoms, respiratory; Asthma; Esophageal pH monitoring; Manometry.

Objective: To evaluate the impact of thoracic radiotherapy on respiratory function and exercise capacity in patients with breast cancer. Methods: Breast cancer patients in whom thoracic radiotherapy was indicated after surgical treatment and chemotherapy were submitted to HRCT, respiratory evaluation, and exercise capacity evaluation before radiotherapy and at three months after treatment completion. Respiratory muscle strength testing, measurement of chest wall mobility, and complete pulmonary function testing were performed for respiratory evaluation; cardiopulmonary exercise testing was performed to evaluate exercise capacity. The total radiotherapy dose was 50.4 Gy (1.8 Gy/fraction) to the breast or chest wall, including supraclavicular lymph nodes (SCLN) or not. Dose-volume histograms were calculated for each patient with special attention to the ipsilateral lung volume receiving 25 Gy (V25), in absolute and relative values, and mean lung dose. Results: The study comprised 37 patients. After radiotherapy, significant decreases were observed in respiratory muscle strength, chest wall mobility, exercise capacity, and pulmonary function test results (p < 0.05). DLCO was unchanged. HRCT showed changes related to radiotherapy in 87% of the patients, which was more evident in the patients submitted to SCLN irradiation. V25% significantly correlated with radiation pneumonitis. Conclusions: In our sample of patients with breast cancer, thoracic radiotherapy seemed to have caused significant losses in respiratory and exercise capacity, probably due to chest wall restriction; SCLN irradiation represented an additional risk factor for the development of radiation pneumonitis.

Keywords: Breast neoplasms; Radiotherapy; Radiation pneumonitis; Respiratory function tests; Exercise test.

We report the case of a patient with chronic obstructive pulmonary disease and bronchiectasis, chronically using corticosteroids, who acquired pulmonary nocardiosis, which presented as multiple cavitated nodules. The principal symptoms were fever, dyspnea and productive cough with purulent sputum. Chest X-ray and computed tomography of the chest revealed nodules, some of which were cavitated, in both lungs. Sputum smear microscopy and culture revealed the presence of Nocardia spp. The patient was treated with imipenem and cilastatin, which produced an excellent clinical response.

Keywords: Pulmonary disease, chronic obstructive; Nocardia infections; Bronchiectasis.