Search

Silvia Maria Doria da Silva, Ilma Aparecida Paschoal, Eduardo Mello De Capitani, Marcos Mello Moreira, Luciana Campanatti Palhares, Mônica Corso PereiraPneumology Service, Department of Internal Medicine, School of Medical Sciences, State University of Campinas (UNICAMP), Campinas, São Paulo, BrazilBackground: Computed tomography (CT) phenotypic characterization helps in understanding the clinical diversity of chronic obstructive pulmonary disease (COPD) patients, but its clinical relevance and its relationship with functional features are not clarified. Volumetric capnography (VC) uses the principle of gas washout and analyzes the pattern of CO2 elimination as a function of expired volume. The main variables analyzed were end-tidal concentration of carbon dioxide (ETCO2), Slope of phase 2 (Slp2), and Slope of phase 3 (Slp3) of capnogram, the curve which represents the total amount of CO2 eliminated by the lungs during each breath.Objective: To investigate, in a group of patients with severe COPD, if the phenotypic analysis by CT could identify different subsets of patients, and if there was an association of CT findings and functional variables.Subjects and methods: Sixty-five patients with COPD Gold III–IV were admitted for clinical evaluation, high-resolution CT, and functional evaluation (spirometry, 6-minute walk test [6MWT], and VC). The presence and profusion of tomography findings were evaluated, and later, the patients were identified as having emphysema (EMP) or airway disease (AWD) phenotype. EMP and AWD groups were compared; tomography findings scores were evaluated versus spirometric, 6MWT, and VC variables.Results: Bronchiectasis was found in 33.8% and peribronchial thickening in 69.2% of the 65 patients. Structural findings of airways had no significant correlation with spirometric variables. Air trapping and EMP were strongly correlated with VC variables, but in opposite directions. There was some overlap between the EMP and AWD groups, but EMP patients had signicantly lower body mass index, worse obstruction, and shorter walked distance on 6MWT. Concerning VC, EMP patients had signicantly lower ETCO2, Slp2 and Slp3. Increases in Slp3 characterize heterogeneous involvement of the distal air spaces, as in AWD.Conclusion: Visual assessment and phenotyping of CT in COPD patients is feasible and may help identify functional and clinically different subsets of patients. VC may provide useful information about the heterogeneous involvement of lung structures in COPD.Keywords: chronic obstructive lung disease, emphysema, volumetric capnography, bronchiectasis, computed tomography

Liliani de Fátima Veronez,1 Monica Corso Pereira,2 Silvia Maria Doria da Silva,2 Luisa Affi Barcaui,2 Eduardo Mello De Capitani,2 Marcos Mello Moreira,2 Ilma Aparecida Paschoalz2 1Department of Physical Therapy, University of Votuporanga (Educational Foundation of Votuporanga), Votuporanga, 2Department of Internal Medicine, School of Medical Sciences, State University of Campinas (UNICAMP), Campinas, Sao Paulo, BrazilBackground: Obstructive lung diseases of different etiologies present with progressive peripheral airway involvement. The peripheral airways, known as the silent lung zone, are not adequately evaluated with conventional function tests. The principle of gas washout has been used to detect pulmonary ventilation inhomogeneity and to estimate the location of the underlying disease process. Volumetric capnography (VC) analyzes the pattern of CO2 elimination as a function of expired volume.Objective: To measure normalized phase 3 slopes with VC in patients with non-cystic fibrosis bronchiectasis (NCB) and in bronchitic patients with chronic obstructive pulmonary disease (COPD) in order to compare the slopes obtained for the groups.Methods: NCB and severe COPD were enrolled sequentially from an outpatient clinic (Hospital of the State University of Campinas). A control group was established for the NCB group, paired by sex and age. All subjects performed spirometry, VC, and the 6-Minute Walk Test (6MWT). Two comparisons were made: NCB group versus its control group, and NCB group versus COPD group. The project was approved by the ethical committee of the institution. Statistical tests used were Wilcoxon or Student’s t-test; P

Introduction: Studies suggest peripheral airway abnormalities in Pulmonary Arterial Hypertension (PAH). Impulse Oscillometry (IOS) is a noninvasive and sensitive technique for assessing the small airways. It evaluates the impedance of the respiratory system ‒ Resistance (R) and reactance (X) ‒ to a pulse of sound waves sent to the lungs, in a range of frequencies (5‒20 Hz)., Method: Resistance variables: R5, R20, R5-R20 and reactance variables: AX (reactance area) and Fres (resonance frequency). The aim is to evaluate R and X in patients with idiopathic PAH (IPAH) and to investigate whether there is a correlation between IOS and spirometry., Results: Thirteen IPAH patients and 11 healthy subjects matched for sex and age underwent IOS and spirometry. IPAH patients had lower FVC and FEV 1 values (p < 0.001), VEF 1 /CVF (p = 0.049) and FEF 25-75 (p = 0.006) than healthy patients. At IOS, IPAH patients showed lower tidal volumes and higher AX (p < 0.05) compared to healthy individuals, and 53.8 of patients had R5-R20 values ≥ 0.07 kPa/L/s. Correlation analysis: X5, AX, R5-R20 and Fres showed moderate correlation with FVC (p = 0.036 r = 0.585, p = 0.001 r = -0.687, p = 0.005 r = -0.726 and p = 0.027 r = -0.610); Fres (p = 0.012 r = -0.669) and AX (p = 0.006 r = -0.711) correlated with FEV 1 ; [R5 and R20, (R5-R20)] also correlated with FEV 1 (p < 0.001 r = -0.573, p = 0.020 r = -0.634 and p = 0.010 r = -0.683, respectively) in the IPAH group. There were also moderate correlations of FEF 25-75 % with Z5 (p = 0.041), R5 (p = 0.018), Fres (p = 0.043) and AX (p = 0.023)., Discussion: Patients showed changes suggestive of increased resistance and reactance in the IOS compared to healthy individuals, and the IOS findings showed a good correlation with spirometry variables., Competing Interests: Declaration of Competing Interest The authors declare no conflicts of interest. The present work was carried out with the support of the Coordination for the Improvement of Higher Education Personnel ‒ Brazil (CAPES) ‒ funding code 001., (Copyright © 2023 HCFMUSP. Published by Elsevier España, S.L.U. All rights reserved.)

Background: Bronchiectasis is frequently identified in patients with COPD, especially in severe patients, but the relevance of this finding remains unclear. We aimed to investigate the factors that would increase the chance of having bronchiectasis in patients with severe COPD., Methods: This is an analytical, observational, cross-sectional study. Patients with severe COPD with (BC group) and without bronchiectasis (NBC group) were clinically evaluated and performed spirometry, 6-minute walk test (6MWT), volumetric capnography (VCap) and high resolution computed tomography (CT). CT was scored for the findings, and multiple linear regression was performed to identify variables related to the score's severity and logistic regression in order to identify factors that could be associated with the presence of bronchiectasis., Results: There was no significant difference between BC and NBC groups regarding clinical variables, except in the smoking load, which was lower in the BC group. In functional evaluation, NBC patients walked shorter distances in 6MWT (P < 0.005). In the BC group the distribution of CT findings was mostly bilateral and in lower lobes. Using the multiple linear regression analysis within the BC group, we found that the higher the bronchiectasis score, the higher ΔSpO2 during the 6MWT and the lower the FVC. The chance of having bronchiectasis was 4.78 times higher in the presence of positive isolates (sputum) (CI 1.35-16.865; P = 0.023). The higher the distance covered (6MWT) and Slp3 (VCap), (OR 1.01, CI 1.004; 1.0202, P = 0.0036; OR 1.04, CI 1.003; 1.077; P = 0.036), the greater are likelihood of bronchiectasis., Conclusions: In patients with COPD and bronchiectasis, higher CT scores were associated with worse lung function and a greater drop in oxygenation during exercise., Competing Interests: None

Background: Elderly people are more likely to suffer severe chronic obstructive pulmonary disease (COPD) and require long-term home oxygen therapy (LTOT) as part of their treatment. LTOT has advantages such as improvement in symptoms, but there are also disadvantages such as physical barriers, psychosocial barriers and emotional challenges. The aim of this study is to understand the experiences of elderly people with COPD using LTOT with respect to their feelings attributed to therapy., Methods: Qualitative study. Seven semidirected interviews were conducted with patients with a confirmed COPD diagnosis who used LTOT and were treated at an outpatient service. The content analysis technique was applied with the support of WebQDA software 2.0., Results: Three categories emerged that were associated with the use of LTOT: (1) poor self-image; (2) feelings of sadness and (3) the impact of LTOT on others such as family and friends., Conclusions: LTOT in elderly people with COPD was associated with a poor self-image, feelings of sadness and impacted on others apart from the patient. When LTOT is prescribed, healthcare practitioners should proactively address these concerns to minimise the negative biopsychosocial experiences caused by LTOT., (© 2022. The Author(s).)

On March 24, 2020, a partial lockdown was decreed in the state of São Paulo, Brazil, as a measure to hinder the spread of COVID-19, which consisted in prohibiting crowding and advising people to stay home, except for urgent or extremely necessary matters. Based on studies performed in other countries, this study aims to assess the impacts of the lockdown on the air quality of five cities in the state of São Paulo. Our study was conducted by using particulate matter and nitrogen dioxide as air quality indicators, and by correlating the contaminants concentrations with weather data. The results showed an increase in these contaminants in all cities within the first weeks after the lockdown compared with the weeks before the decree and with the same period in previous years. This result is inconsistent with the literature. Therefore, a secondary goal was set to investigate the possible cause (or causes) of such deterioration in air quality, which led to the increased number of wildfires. The anomalous dry weather favored the burning of vegetation in agricultural rural areas and in small, vegetated areas near the municipalities, and limited pollution scavenging by rainfall, both of which contributed to higher pollution concentration. We hypothesize the possible effects of worse air quality on the aggravation of COVID-19, but further research is necessary to obtain a complete assessment.

Introduction: Inspiratory muscle training (IMT) has been shown to be an efficient method of improving exercise tolerance and inspiratory and expiratory muscle strength in several diseases. The effects of IMT on patients with sickle cell anemia (SCD) are relatively unknown. Our study aimed to evaluate the effects of IMT on adult SCD patients, regarding respiratory muscle strength (RMS) variables, lung function, exercise tolerance, blood lactation concentration, limitation imposed by dyspnea during daily activities and impact of fatigue on the quality of life., Methods: This was a randomized single-blind study, with an IMT design comprising true load (TG) and sham load (SG) groups. Initial assessment included spirometry, volumetric capnography (VCap) and measurement of RMS by maximal inspiratory and expiratory pressure (PImax and PEmax). The Medical Research Council dyspnea scale and modified fatigue impact scale were also applied and blood lactate concentration was measured before and after the 6-minute walk test. After this initial assessment, the patient used the IMT device at home daily, returning every 6 weeks for RMS reassessment. Both groups used the same device and were unaware of which group they were in. After a period totaling 18 weeks, patients underwent the final evaluation, as initially performed., Results: Twenty-five patients in total participated until the end of the study (median age 42 years). There were no significant differences between TG and SG based on age, sex, body mass index or severity of genotype. At the end of the training, both groups showed a significant increase in PEmax and PImax, improvement in Vcap and in exercise tolerance and dyspnea reduction while performing daily life activities. The same was observed in patients grouped according to disease severity (HbSS and HbSβ 0 vs HbSC and HbSβ + ), without differences between groups., Conclusion: Home-based inspiratory muscle training benefits outpatients with SCD, including the sham load group., Trial Registration: http://www.ensaiosclinicos.gov.br; registration number: RBR-6g8n92., (Copyright © 2020 Associação Brasileira de Hematologia, Hemoterapia e Terapia Celular. Published by Elsevier España, S.L.U. All rights reserved.)

Background: The mechanism of exercise limitation in idiopathic pulmonary arterial hypertension (IPAH) is not fully understood. The role of hemodynamic alterations is well recognized, but mechanical, ventilatory and gasometric factors may also contribute to reduction of exercise capacity in these individuals., Objective: To investigate whether there is an association between ventilatory pattern and stress Doppler echocardiography (SDE) variables in IPAH patients., Design and Setting: Single-center prospective study conducted in a Brazilian university hospital., Methods: We included 14 stable IPAH patients and 14 age and sex-matched controls. Volumetric capnography (VCap), spirometry, six-minute walk test and SDE were performed on both the patients and the control subjects. Arterial blood gases were collected only from the patients. The IPAH patients and control subjects were compared with regard to the abovementioned variables., Results: The mean age of the patients was 38.4 years, and 78.6% were women. The patients showed hypocapnia, and in spirometry 42.9% presented forced vital capacity (FVC) below the lower limit of normality. In VCap, IPAH patients had higher respiratory rates (RR) and lower elimination of CO2 in each breath. There was a significant correlation between reduced FVC and the magnitude of increases in tricuspid regurgitation velocity (TRV). In IPAH patients, VCap showed similar tidal volumes and a higher RR, which at least partially explained the hypocapnia., Conclusions: The patients with IPAH showed hypocapnia, probably related to their higher respiratory rate with preserved tidal volumes; FVC was reduced and this reduction was positively correlated with cardiac output.

Pulmonary arterial hypertension impairs exercise tolerance and daily physical activity. Aside from the hemodynamic limitations, physical, cognitive, and emotional factors may play a relevant and as yet unexplored role. We investigated whether there is an association between the physical activity level and psychological disorders, health-related quality of life, and daily activities. We also searched for an association of the physical activity level with clinical factors and functional capacity. This was an analytical, cross-sectional, observational study conducted in a Brazilian University Hospital. Twenty stable pulmonary arterial hypertension subjects wore an accelerometer for a week and completed an activity diary. They answered the quality of life questionnaire (SF-36), as well as the Hospital Anxiety and Depression scale, and the Manchester Respiratory Activities of Daily Living questionnaire. Transthoracic echocardiography, the six-minute walk test, the one-minute sit-to-stand test, and spirometry were performed. For statistical analysis, we used Chi-square tests or Fisher's test as appropriate and the Mann-Whitney test to compare numerical values between two groups. The relationship between the parameters was assessed using the Spearman correlation test. The mean age was 44.3 years, 80% were women, 80% had idiopathic pulmonary arterial hypertension, and 20% had connective tissue disease. The mean daily step count was 4280 ± 2351, and the mean activity time was 41.6 ± 19.3 min. The distance covered (six-minute walk test) was 451.5 m, and the number of movements (one-minute sit-to-stand test) was 23.8. Thirty percent scored positive for anxiety, and 15% for depression (Hospital Anxiety and Depression scale). There was a significant correlation between accelerometer data and walking distance (six-minute walk test), number of movements (one-minute sit-to-stand test), level of daily physical activity (Manchester Respiratory Activities of Daily Living questionnaire), and depression symptoms. Our findings support the hypothesis that other aspects beyond physical and hemodynamic ones might impact the daily physical activity of patients with pulmonary arterial hypertension., (© The Author(s) 2021.)

Introduction: Cystic fibrosis (CF) presents with progressive and chronic deterioration of lung function due to inflammation and colonization/infection of the lungs. This study evaluated spirometry and colonization/infection with Staphylococcus aureus and/or Pseudomonas aeruginosa over a 24-month follow-up period., Methods: A total of 52 CF patients were studied with spirometry: forced vital capacity (FVC), forced expiratory volume in one second of FVC (FEV 1 ), FEV 1 /FVC and forced expiratory flow between 25% and 75% of FVC (FEF 25 -75% ). Colonization/infection was evaluated as predominantly S. aureus, predominantly P. aeruginosa or concomitance of these microorganisms., Results: In CF, there was a higher prevalence of p.Phe508del/p.Phe508del genotype (16/52; 30.8%) and female gender (33/52; 63.5%). Spirometry (% predicted) markers worsened for the following groups over the 24-month period: (i) male: FVC, FEV 1 , FEV 1 /FVC, FEF 25-75% ; (ii) female: FVC%, FEV 1 , (iii) predominantly S. aureus: FVC, FEV 1 , FEV 1 /FVC, FEF 25-75% ; (iv) predominantly P aeruginosa: FEV 1 /FVC; (v) concomitant S. aureus and P. aeruginosa: FVC, FEV 1 . Age correlated with reduction of FVC(Liter) (Rho = -0.50) and FEV 1 (Liter) (Rho = -0.46). Pancreatic insufficiency and severe cystic fibrosis transmembrane regultador (CFTR) mutations were associated with deteriorating lung function., Conclusion: In CF, deterioration of lung function as evaluated by spirometry was continuous and varied according to sex, pancreatic insufficiency, and severe CFTR mutations. No differences were observed between groups in terms of predominant type of bacteria, but the reduction of spirometry parameters was significant in the predominantly S. aureus and concomitant infection groups.

Objective: To compare the values of the markers for volumetric capnography and spirometry and their ability to classify children and adolescents with asthma, cystic fibrosis (CF), and healthy controls., Methods: This was a cross-sectional study that included 103 patients with controlled persistent allergic asthma, 53 with CF and a healthy control group with 40 volunteers (aged 6 to 15 years), of both sexes. The individuals underwent volumetric capnography and spirometry., Results: Phase III slope (SIII), SIII standardized by exhaled tidal volume (SIII/TV) and capnographic index (SIII/SII)×100 (KPIv) were different among the three groups assessed, with highest values for CF. The relation between the forced expiratory volume in one second and the forced vital capacity (FEV 1 /FVC) was the only spirometric marker that presented difference on the three groups. On individuals with normal spirometry, KPIv and FEV 1 /FVC were different among the three groups. The ROC curve identified the individuals with asthma or CF from the control group, both through volumetric capnography (better to identify CF in relation to the control using KPIv) and through spirometry (better to identify asthma in relation to the control). KPIv was the best parameter to distinguish asthma from CF, even in individuals with normal spirometry., Conclusion: Volumetric capnography and spirometry identified different alterations in lung function on asthma, CF, and healthy controls, allowing the three groups to be distinguished., (Copyright © 2018 Sociedade Brasileira de Pediatria. Published by Elsevier Editora Ltda. All rights reserved.)

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.

Objective: Cystic fibrosis diagnosis is dependent on the chloride ion concentration in the sweat test (≥60mEq/mL - recognized as the gold standard indicator for cystic fibrosis diagnosis). Moreover, the salivary glands express the CFTR protein in the same manner as sweat glands. Given this context, the objective was to verify the correlation of saliva chloride concentration and sweat chloride concentration, and between saliva sodium concentration and sweat sodium concentration, in patients with cystic fibrosis and healthy control subjects, as a tool for cystic fibrosis diagnosis., Methods: There were 160 subjects enrolled: 57/160 (35.70%) patients with cystic fibrosis and two known CFTR mutations and 103/160 (64.40%) healthy controls subjects. Saliva ion concentration was analyzed by ABL 835 Radiometer ® equipment and, sweat chloride concentration and sweat sodium concentration, respectively, by manual titration using the mercurimetric procedure of Schales & Schales and flame photometry. Statistical analysis was performed by the chi-squared test, the Mann-Whitney test, and Spearman's correlation. Alpha=0.05., Results: Patients with cystic fibrosis showed higher values of sweat chloride concentration, sweat sodium concentration, saliva chloride concentration, and saliva sodium concentration than healthy controls subjects (p-value<0.001). The correlation between saliva chloride concentration and sweat chloride concentration showed a positive Spearman's Rho (correlation coefficient)=0.475 (95% CI=0.346 to 0.587). Also, the correlation between saliva sodium concentration and sweat sodium concentration showed a positive Spearman's Rho=0.306 (95% CI=0.158 to 0.440)., Conclusions: Saliva chloride concentration and saliva sodium concentration are candidates to be used in cystic fibrosis diagnosis, mainly in cases where it is difficult to achieve the correct sweat amount, and/or CFTR mutation screening is difficult, and/or reference methods for sweat test are unavailable to implement or are not easily accessible by the general population., (Copyright © 2018 Sociedade Brasileira de Pediatria. Published by Elsevier Editora Ltda. All rights reserved.)

Background: Excess trunk body fat in obese individuals influences respiratory physiological function. The aims of this study were to compare volumetric capnography findings (VCap) between severely obese patients and normal-weight subjects and to assess whether there is any association between neck circumference (NC), waist-hip ratio (WHR) and VCap among grade III obese individuals., Design and Setting: Analytical observational case-matched cross-sectional study, University of Campinas., Methods: This cross-sectional study compared VCap variables between 60 stage III obese patients and 60 normal-weight individuals., Results: In comparison with the normal-weight group, obese patients presented higher alveolar minute volume (8.92 ± 4.94 versus 6.09 ± 2.2; P = < 0.0001), CO2 production (278 ± 91.0 versus 209 ± 60.23; P < 0.0001), expiratory tidal volume (807 ± 365 versus 624 ± 202; P = 0.005), CO2 production per breath (21.1 ± 9.7 versus 16.7 ± 6.16; P = 0.010) and peak expiratory flow (30.9 ± 11.9 versus 25.5 ± 9.13; P = 0.004). The end-expiratory CO2 (PetCO2) concentration (33.5 ± 4.88 versus 35.9 ± 3.79; P = 0.013) and the phase 3 slope were normalized according to expired tidal volume (0.02 ± 0.05 versus 0.03 ± 0.01; P = 0.049) were lower in the obese group., Conclusions: The greater the NC was, the larger were the alveolar minute volume, anatomical dead space, CO2 production per minute and per breath and expiratory volume; whereas the smaller were the phase 2 slope (P2Slp), phase 3 slope (P3Slp) and pressure drop in the mouth during inspiration.

Nontuberculous mycobacteria (NTM) have been well established as an opportunistic pathogenic bacterial group for cystic fibrosis (CF) patients, with a prevalence ranging from 3% to 23% worldwide. A myriad of factors can bias the prevalence rate in different CF centers, especially misdiagnosis as systematic screening for NTM are still lacking in a number of centers. Here, we evaluated the presence and clinical outcomes of NTM isolation in microbiological respiratory cultures from CF patients attending a Brazilian reference center after setting up a systematic diagnostic protocol. Of 117 patients with respiratory samples cultured for NTM research, we found seven patients (6%) with at least one positive result for NTM [four males (57.1%), median age = 21 years (9-58)]. These cases are reported one-by-one. Median FEV1 was 40%, all patients showed signs of lung deterioration, with a median number of pulmonary exacerbations of three per patient/year. However, the impact of NTM isolation remains unclear in our center as all patients were coinfected with other CF respiratory pathogens. Our NTM prevalence assimilates to the lowest levels reported in literature, which is possibly influenced by the routinely applied Bacille Calmette-Guérin vaccine.

Objective: To analyze and compare lung function of obese and healthy, normal-weight children and adolescents, without asthma, through spirometry and volumetric capnography., Methods: Cross-sectional study including 77 subjects (38 obese) aged 5-17 years. All subjects underwent spirometry and volumetric capnography. The evaluations were repeated in obese subjects after the use of a bronchodilator., Results: At the spirometry assessment, obese individuals, when compared with the control group, showed lower values of forced expiratory volume in the first second by forced vital capacity (FEV 1 /FVC) and expiratory flows at 75% and between 25 and 75% of the FVC (p<0.05). Volumetric capnography showed that obese individuals had a higher volume of produced carbon dioxide and alveolar tidal volume (p<0.05). Additionally, the associations between dead space volume and tidal volume, as well as phase-3 slope normalized by tidal volume, were lower in healthy subjects (p<0.05). These data suggest that obesity does not alter ventilation homogeneity, but flow homogeneity. After subdividing the groups by age, a greater difference in lung function was observed in obese and healthy individuals aged >11 years (p<0.05)., Conclusion: Even without the diagnosis of asthma by clinical criteria and without response to bronchodilator use, obese individuals showed lower FEV 1 /FVC values and forced expiratory flow, indicating the presence of an obstructive process. Volumetric capnography showed that obese individuals had higher alveolar tidal volume, with no alterations in ventilation homogeneity, suggesting flow alterations, without affecting lung volumes., (Copyright © 2017 Sociedade Brasileira de Pediatria. Published by Elsevier Editora Ltda. All rights reserved.)

Objective: Quality of life (QOL), sexual satisfaction (SS) and physical performance have been assessed in the management of numerous chronic diseases., Methods: In this study, the following tests and surveys were applied: (i) QOL questionnaire [Cystic Fibrosis Questionnaire (CFQ)]; (ii) SS questionnaire (SSQ) [female sexual quotient (FSQ) and male sexual quotient (MSQ)]; (iii) 6-minute walk test (6MWT). Spearman's correlation was used for comparison between the data; the Mann-Whitney test was applied to analyze the difference between genders. A total of 52 adult patients with CF were included in this study., Results: There was a positive correlation between CFQ domains and SSQ questions. The CFQ showed a positive correlation with peripheral oxygen saturation of hemoglobin (SpO 2 ) and the distance walked in the 6MWT, and a negative correlation with the Borg scale. The SSQ showed positive correlation with the distance walked and a negative correlation with the Borg scale. For some markers evaluated in the 6MWT, there was sometimes association with the evaluated domains and questions. Male patients showed better scores in the emotional CFQ domain, better performance in SSQ and physical performance., Conclusions: There was a correlation between CFQ, SSQ and 6MWT in CF. Finally; we believe that QOL surveys should assess the domain "sexuality" as well as physical performance tests., (Copyright © 2017 Sociedade Portuguesa de Pneumologia. Published by Elsevier España, S.L.U. All rights reserved.)

Background: To assess the quality of sweat test (ST) based on the proportion of sweat sodium and sweat chloride as diagnostic parameter of cystic fibrosis (CF)., Methods: A retrospective study of 5,721 sweat samples and subsequent descriptive analysis were carried out. The test was considered "of good quality" (correct) when: (i) sweat chloride was lower than 60 mEq/L, and sweat sodium was higher than sweat chloride; (ii) sweat chloride was higher than 60 mEq/L, and sweat sodium was lower than sweat chloride., Results: The study included 5,692/5,721 sweat samples of ST which had been requested due to clinical presentations compatible with CF and/or neonatal screenings with altered immunoreactive trypsinogen values. Considering the proportion of sweat sodium and sweat chloride as ST quality parameter, the test was performed correctly in 5,023/5,692 (88.2 %) sweat samples. The sweat chloride test results were grouped into four reference ranges for chloride (i) chloride < 30 mEq/L: 3,651/5,692 (64.1 %); (ii) chloride ≥ 30 mEq/L to < 40 mEq/L: 652/5,692 (11.5 %); (iii) ≥ 40 mEq/L to < 60 mEq/L: 673/5,692 (11.8 %); (iv) ≥ 60 mEq/L: 716/5,692 (12.6 %). In the comparative analysis, there was no association between ST quality and: (i) symptoms to indicate a ST [respiratory (p = 0.084), digestive (p = 0.753), nutritional (p = 0.824), and others (p = 0.136)], (ii) sweat weight (p = 0.416). However, there was a positive association with: (i) gender, (ii) results of ST (p < 0.001), (iii) chloride/sodium ratio (p < 0.001), (iv) subject's age at the time of ST [grouped according to category (p < 0.001) and numerical order (p < 0.001)]. For the subset of 169 patients with CF and two CFTR mutations Class I, II and/or III, in comparative analysis, there was a positive association with: (i) sweat chloride/sodium ratio (p < 0.001), (ii) sweat chloride values (p = 0.047), (iii) subject's age at the time of the ST grouped by numerical order (p = 0.001)., Conclusions: Considering that the quality of ST can be assessed by levels of sweat sodium and sweat chloride, an increasing number of low-quality tests could be observed in our sweat samples. The quality of the test was associated with important factors, such as gender, CF diagnosis, and subjects' age.

Background: Exercise has been studied as a prognostic marker for patients with cystic fibrosis (CF), as well as a tool for improving their quality of life and analyzing lung disease. In this context, the aim of the present study was to evaluate and compare variables of lung functioning. Our data included: (i) volumetric capnography (VCAP) parameters: expiratory minute volume (VE), volume of exhaled carbon dioxide (VCO2), VE/VCO2, ratio of dead space to tidal volume (VD/VT), and end-tidal carbon dioxide (PetCO2); (ii) spirometry parameters: forced vital capacity (FVC), percent forced expiratory volume in the first second of the FVC (FEV1%), and FEV1/FVC%; and (iii) cardiorespiratory parameters: heart rate (HR), respiratory rate, oxygen saturation (SpO2), and Borg scale rating at rest and during exercise. The subjects comprised children, adolescents, and young adults aged 6-25 years with CF (CF group [CFG]) and without CF (control group [CG])., Methods: This was a clinical, prospective, controlled study involving 128 male and female patients (64 with CF) of a university hospital. All patients underwent treadmill exercise tests and provided informed consent after study approval by the institutional ethics committee. Linear regression, Kruskal-Wallis test, and Mann-Whitney test were performed to compare the CFG and CG. The α value was set at 0.05., Results: Patients in the CFG showed significantly different VCAP values and spirometry variables throughout the exercise test. Before, during, and after exercise, several variables were different between the two groups; statistically significant differences were seen in the spirometry parameters, SpO2, HR, VCO2, VE/VCO2, PetCO2, and Borg scale rating. VCAP variables changed at each time point analyzed during the exercise test in both groups., Conclusion: VCAP can be used to analyze ventilatory parameters during exercise. All cardiorespiratory, spirometry, and VCAP variables differed between patients in the CFG and CG before, during, and after exercise.

Objective: To evaluate the risk factors and comorbid conditions associated with the development of pneumonia in patients with acute stroke. To determine the independent predictors of pneumonia., Method: Retrospective study from July to December 2011. We reviewed all medical charts with diagnosis of stroke., Results: 159 patients (18-90 years) were admitted. Prevalence of pneumonia was 32%. Pneumonia was more frequent in patients with hemorrhagic stroke (OR: 4.36; 95%CI: 1.9-10.01, p < 0.001), higher National Institute of Health Stroke Scale (NIHSS) (p = 0.047) and, lower Glasgow Coma Score (GCS) (p < 0.0001). Patients with pneumonia had longer hospitalization (p < 0.0001). Multivariable logistic regression analysis identified NIHSS as an independent predictor of pneumonia (95%CI: 1.049-1.246, p = 0.002)., Conclusion: Pneumonia was associated with severity and type of stroke and length of hospital stay. The severity of the deficit as evaluated by the NIHSS was shown to be the only independent risk factor for pneumonia in acute stroke patients.

Background: There is no consensus regarding obesity repercussions for lung function in children and adolescents. Therefore, the aim of the study was to determine whether obesity is associated with poor physical conditioning and damaged lung function in children and adolescents, and to correlate lung function with six-minute walk test (6MWT) results., Methods: This cross-sectional study included 38 obese subjects of both sexes, ranging between 5 and 17 years of age, as well as 56 control subjects paired by sex and age for the 6MWT, and 39 subjects for spirometry. Subjects performed spirometry according to the guidelines of the American Thoracic Society (ATS) and the European Respiratory Society. The obese group repeated spirometry after receiving bronchodilator (BD) treatments. Physical performance was evaluated via the 6MWT according to ATS guidelines., Results: The obese group demonstrated lower forced expiratory volumes in the first second compared with the control group based on forced vital capacity indices (p < 0.01), findings consistent with airway obstruction in 36.8% of patients in the obese group. Walking distances were shorter in the obese group than in the control group. Changes in lung function did not correlate directly with performance on the 6MWT among obese patients. However, there was a correlation between lung function and variables indicative of effort during exercise., Conclusion: In the present study, the obese group walked shorter distances and demonstrated lower values in some markers of lung function. However, there is no relationship between their physical conditions and these test results. Therefore, we cannot conclusively state that poor physical performance results from damaged pulmonary function.

Background: There are nearly 2000 cystic fibrosis transmembrane regulator (CFTR) mutations that cause cystic fibrosis (CF). These mutations are classified into six classes; on the one hand, the first three classes cause severe disease involvement in early childhood, on the other hand, the Class IV, V and VI mutations cause minor severe disease in the same age. Nowadays, with therapeutic advances in CF management and competence of pediatricians, physicians of adults have to deal with two groups of CF patients: (i) adults diagnosed in childhood with severe mutations and (ii) adults who initiated symptoms in adulthood and with Class IV, V and VI mutations. The aim of this study was to analyze adults from a clinical center, treated as CF disease, screening the CFTR genotype and evaluating the clinical characteristics., Methods: Thirty patients followed as CF disease at the University Hospital were enrolled. After a complete molecular CFTR negative screening and sweat test levels between 40 and 59mEq/L, five patients were characterized as non-CF disease and were excluded. Molecular screening was performed by CFTR gene sequencing/MLPA or by specific mutation screening. Clinical data was obtained from medical records. The patients were divided into three groups: (1) patients with Class I, II and III mutations in two CFTR alleles; (2) genotype with at least one allele of Class IV, V or VI CFTR mutations and, (3) non-identified CFTR mutation+one patient with one allele with CFTR mutation screened (Class I)., Results: There was an association of CFTR class mutation and sodium/chloride concentration in the sweat test (sodium: p=0.040; chloride: p=0.016), onset of digestive symptoms (p=0.012), lung function parameter (SpO2 - p=0.016), Bhalla score (p=0.021), age at diagnosis (p=0.008) and CF-related diabetes (p=0.029). There was an association between Pseudomonas aeruginosa chronic colonization (as clinical marker for the lung disease status) and lung impairment (FEV1% - p=0.027; Bhalla score - p=0.021), CF-related diabetes (p=0.040), chloride concentration in the sweat test (p=0.040) and chronic infection by microorganisms (Staphylococcus aureus - p=0.039; mucoid P. aeruginosa - p=0.001). There is no positive association with the status of other clinical markers and the CFTR genotype groups. For clinical association with pancreatic insufficiency (as clinical marker for digestive symptoms), no association was related., Conclusion: The adults with CF diagnosed by sweat test have specific clinical and genotypic characteristics, being a population that should be studied to cause better future management. Some patients treated as CF disease by clinical symptoms, showed no disease, taking into account the sweat test and complete exon sequencing/MLPA screening., (Copyright © 2014. Published by Elsevier B.V.)

Objective: Evaluate SLE stable patients, without overt respiratory compromise, by means of 6MWT., Casuistic and Methods: Forty-five stable SLE patients were enrolled. The ATS/ERS protocol for 6MWT, was used and two parameters with cut-off points were chosen., Results: Forty-two patients were women. The mean age was 39 ± 11.4 years; mean duration of disease, 121 ± 93.1 months; mean value of MRC, 2 ± 0; mean FVC, 85.9 ± 34.2%; mean FEV1, 67.5 ± 21.6%; mean MIP, 82 ± 58.4%; mean MEP, 78 ± 37.3%; mean heart rate at rest, 75 ± 12.8 bpm; mean respiratory rate at rest, 19 ± 5.3 bpm; mean 6MWD, 478 ± 82 m; mean SpO2 at rest was 98 ± 0.8%; mean fall in SpO2, 4 ± 6 points. When the study population was divided according to the 400-m walk distance cut-off value, the heart rate immediately before the test was significant lower in those participants who walked less than 400 m (p = 0.0043), just like the value of Borg scale (p = 0.0036); according to the presence of saturation ≥ 4, heart rate at the end of the test was significantly higher in those participants who were showing desaturation (p = 0.0170); MEP (p = 0.0282) and 6MWD (p = 0.0291) were significantly lower, and MIP showed a tendency towards being smaller (p = 0.0504). FVC < normal inferior limit was significantly associated with the group with desaturation (p = 0.0274)., Conclusion: Compared to 6MWD, desaturation was better suited to find the patients with the most compromised indexes in respiratory function tests.

Objective: To determine the probability of oxygen desaturation in healthy individuals undergoing the incremental shuttle walk test (ISWT)., Methods: We enrolled 83 healthy subjects: 55 males (including 1 smoker) and 28 females. We determined pre-ISWT FEV1, FEV6, HR and SpO2, as well as post-ISWT HR and SpO2., Results: Mean values overall were as follows: age, 35.05 ± 12.53 years; body mass index, 24.30 ± 3.47 kg/m2; resting HR, 75.12 ± 12.48 bpm; resting SpO2, 97.96 ± 1.02%; FEV1, 3.75 ± 0.81 L; FEV6, 4.45 ± 0.87 L; FEV1/FEV6 ratio, 0.83 ± 0.08 (no restriction or obstruction); incremental shuttle walk distance, 958.30 ± 146.32 m; post-ISWT HR, 162.41 ± 18.24 bpm; and post-ISWT SpO2, 96.27 ± 2.21%. In 11 subjects, post-ISWT SpO2 was higher than was pre-ISWT SpO2. In 17 subjects, there was a 4% decrease in SpO2 after the ISWT. There were no statistically significant differences between the groups with and without post-ISWT oxygen desaturation in terms of age, gender, FEV1, FEV6, FEV1/FEV6, pre-ISWT SpO2, incremental shuttle walk distance, HR, or percentage of maximal HR. In the individuals with post-ISWT oxygen desaturation, the body mass index was higher (p = 0.01) and post-ISWT SpO2 was lower (p = 0.0001)., Conclusions: Healthy individuals can present oxygen desaturation after the ISWT. Using the ISWT to predict subtle respiratory abnormalities can be misleading. In healthy subjects, oxygen desaturation is common after the ISWT, as it is during any intense physical activity.

Background: Saliva and sweat are modified by cystic fibrosis (CF). In both cases the chloride and sodium ion concentrations for healthy subjects and CF patients differ, this representing a possible alternative tool for CF diagnosis. In this context, the aim of this study was to compare the concentrations of these ions in saliva samples taken from CF patients and healthy subjects., Methods: A case-control study was carried out at a university CF center, in which the saliva samples were analyzed on an ABL 835 Radiometer® to determine the ion concentration., Results: For the CF patients (n = 80) the values for the biochemical parameters of chloride, potassium and sodium ion concentration were higher (p < 0.009) and the volume and pH of the saliva were lower than in the case of healthy subjects (p < 0.009). For the healthy subjects group (n = 84) versus CF patients, according to the ROC curve, the values for sodium were: cutoff: 13.5 mmol/L, sensitivity: 73.4%, specificity: 70.6%; and for chloride: cutoff: 20 mmol/L, sensitivity: 68.1%, specificity: 72.9%., Conclusions: The chloride and sodium concentrations in the saliva samples were higher for CF patients in comparison with healthy subjects. Thus, saliva as a tool for CF diagnosis can be considered a new challenge, and a population study including patients in all age classes needs to be performed, in different countries over the world, to extend the database to include a broad spectrum of information in order to identify normal ion concentration ranges for CF patients according to age, genotype and environment., Virtual Slides: The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/2614233148750145.

Introduction: Poorly reversible airflow obstruction may or may not be related to smoking., Objectives: To describe patients with severe obstructive lung disease including etiology, imaging, functional aspects, systemic manifestations, and the pattern of bronchodilator response., Methods: Sixty-eight patients (age 55.9±13.7 years, FEV(1) [forced expiratory volume in one second] 31.9±10.2% predicted) underwent spirometry, evaluation of body mass composition, 6-minute walk test, X-ray, thorax high-resolution CT scanning, and clinical evaluation., Results: Of 68 patients enrolled, 37 had chronic obstructive pulmonary disease (COPD) and 31, extensive bronchiectasis. Among COPD patients the CT scans showed emphysema in 78.4%, and bronchiectasis in 48.6%. There were no significant differences between smokers and non-smokers, except for vital capacity, significantly smaller in non-smokers (p<0.001). We found 29 and 20 volume responders (VR) according to Paré et al. (FEV(1)/FVC>1=flow responder or <1=VR) and ATS/ERS criteria, respectively. According to Paré et al. criteria, there were 18 patients with FEV(1)<30% predicted among 29 VR, and 12 with FEV(1)<30% predicted among 39 without volume response (p=0.0101)., Conclusions: In patients with severe obstruction, smoking does not appear to be relevant in determining functional or systemic differences, and Paré et al. criteria can detect more VR. Bronchiectasis is a common finding in severe COPD., (Copyright © 2012 Sociedade Portuguesa de Pneumologia. Published by Elsevier España. All rights reserved.)

Objective: To compare spirometry and volumetric capnography (VCap) to determine if the capnographic values add more information about early lung disease in cystic fibrosis (CF) patients., Methods: This was a cross-sectional study involving CF patients: Group I (42 patients, 6-12 years of age); and Group II (22 patients, 13-20 years of age). The corresponding control groups were comprised of 30 and 50 healthy subjects, respectively. Forced vital capacity (FVC), forced expiratory volume in one second (FEV(1)), and the FEV(1)/FVC ratio was determined by spirometry. Using VCap, we measured peripheral oxygen saturation (SpO(2)), respiratory rate (RR), inspiratory time (IT), expiratory time (ET), and the phase III slope normalized by expiratory volume (phase III slope/Ve)., Results: In comparison with control groups, all CF patients presented higher phase III slope/Ve values (p < 0.001) independent of the pulmonary disease stage. The phase III slope/Ve was significantly higher in the 24 patients who presented normal spirometry results (p = 0.018). The Group II patients showed lower FVC, FEV(1), FEV(1)/FVC (p < 0.05), and also lower SpO(2) values (p < 0.001) when compared with Group I patients. In comparison with Control Group II, the Group II patients presented higher RR (p < 0.001), and lower IT and ET values (p < 0.001)., Conclusions: Compared to the controls, all studied CF patients showed an increase in phase III slope/Ve values. VCap identified the heterogeneity of the ventilation distribution in the peripheral airways of CF patients who presented normal spirometry.

Background: Immunosuppression is the leading cause of recurrent sinus infections after hematopoietic stem cell transplant (HSCT), with increased incidence of sinusitis in patients with chronic graft versus host disease (GVHD). Histological descriptions of the oral mucosa, lung ciliary epithelium, and intestinal mucosa related to HSCT have been described. However, few have described the nasal mucosa. We, therefore, sought to elucidate the histological and ultrastructural features of the nasal mucosa in patients after HSCT to better understand the pathophysiology of the immune response., Methods: Uncinate processes from 24 HSCT patients and 12 immunocompetent patients were subjected to histological analyses via light and transmission electron microscopy (TEM)., Results: TEM revealed aberrant cilia structure, altered mitochondria quantity, microvilli, and cytoplasm vacuolization. All HSCT patients with rhinosinusitis had significant loss or absence of cilia (p = 0.018). Apoptotic bodies were increased and Goblet cells decreased in nasal epithelium from patients with chronic GVHD (p = 0.04)., Conclusion: This tissue destruction likely enhances pathogen penetration resulting in recurrent infection.

Introduction: Experimental studies on pulmonary embolism (PE) are usually performed under mechanical ventilation. Most patients with suspicion of PE enter the Emergency Services in spontaneous breathing and environmental air. Thus, under these conditions, measurements of hemodynamic, gasometric and capnographic variables contribute largely to a more specific comprehension of cardiopulmonary and gasometric alterations in the acute phase of the disease. Studies which evaluated animals under conditions are lacking., Objective: This study aimed to submit animals under spontaneous ventilation and without supplemental oxygen to PE., Methods: PE was induced in six pigs using autologous blood clots, and cardiorespiratory and gasometric records were performed before and after PE. The values of "near fatal" mean pulmonary arterial pressure (MPAP) were previously determined., Results: The presence of obstructive shock could be evidenced by increased MPAP (from 17.8 ± 3.5 to 41.7 ± 3.3 mmHg) (P<0.0001) and decreased cardiac output (from 4.9 ± 1.0 to 2.7 ± 1.0 L/min) (P<0.003). Consequently, metabolic acidosis occurred (Lac art) (from 2.4 ± 0.6 to 5.7 ± 1.8 mmol/L)(P<0.0001). It was observed hypoxemia (from 73.5 ± 12.7 to 40.3 ± 4.6 mmHg) (P<0.0001); however, PaCO2 did not vary (from 44.9 ± 4.4 to 48.2 ± 6.0 mmHg) (NS). There were significant increases in both P(a-et)CO2 (from 4.8 ± 2.8 to 37.2 ± 5.8 mmHg) and P(A-a)O2 (from 8.2 ± 8.9 to 37.2 ± 10.3 mmHg) (both P<0.0001). There was also a significant increase in the total alveolar minute volume (from 4.0 ± 0.9 to 10.6 ± 2.9 L/min) (P<0.0001)., Conclusions: In this model, the near fatal MPAP was from 2 to 2.5 times the basal MPAP; and the capnographic variables, associated with arterial and venous gasometry, showed effective in discriminating an acute obstructive profile.

This is the first report of a patient submitted to chemical thrombolysis due to massive pulmonary embolism (PE) during the postoperative period of neurosurgery, in whom due to the lack of adequate clinical conditions, no imaging assessment was performed. Clinical, gasometric and capnographic data allowed the decision to perform the thrombolysis with safety. The P(a-et)CO2 gradient decreased from 46.4 mmHg to 11.8 mmHg (normal < 5 mmHg) and the end-tidal alveolar dead space fraction decreased from 0.85 to 0.37 (normal < 0.15) from the pre-thrombolysis period to the 7th day post-thrombolysis. We conclude that the volumetric capnography (VC) was useful in the patient's diagnosis and clinical follow-up.

Summary Background: Health-related quality of life (HRQOL) measurements provide valuable information about the psychological and social impact of treatment on patients with cystic fibrosis (CF). This study evaluated the HRQOL of Brazilian patients with CF and assessed the changes in HRQOL domains over 1 year after dornase alfa (Pulmozyme) introduction., Patients and Methods: One hundred fifty-six stable patients with CF and 89 caregivers answered the Portuguese-validated version of the Cystic Fibrosis Questionnaire-Revised (CFQ-R) at baseline (T(0)), and at 3 (T(1)), 6 (T(2)), 9 (T(3)), and 12 (T(4)) months of follow-up. Eighteen patients were excluded because they did not fulfill the inclusion criteria. The patients were analyzed in two groups: those aged 6-11 years and those aged 14 years and older. ANOVA for observed repeated results and the last observation carried forward (LOCF) method for missing data were used for the statistical analysis., Results: After 1 year of follow-up, there was significant improvement in respiratory symptoms (T(4) - T(0) = 8.1; 95% confidence interval (95% CI) = [2.1;14.0]; effect size (ES) = 0.35; P < 0.001), Emotional Functioning (T(4) - T(0) = 5.6; 95% CI = [1.1;10.1]; ES = 0.31; P < 0.05), Social Functioning (T(4) - T(0) = 6.0; 95% CI = [1.3;11.7]; ES = 0.31; P < 0.05), Body Image (T(4) - T(0) = 11.9; 95% CI = [4.1;19.7]; ES = 0.42; P < 0.05), and Treatment Burden (T(4) - T(0) = 5.3; 95% CI = [0.3;10.3]; ES = 0.24; P < 0.05) domains in the younger group. A significant improvement in Role Functioning (T(4) - T(0) = 6.1; 95% CI = [1.1;11.1]; ES = 0.40; P < 0.05), Body Image (T(4) - T(0) = 12.6; 95% CI = [3.5;21.7]; ES = 0.46; P < 0.05), and Weight (T(4) - T(0) = 11.7; 95% CI = [1.8;21.6]; ES = 0.40; P < 0.05) was obtained in the older group. The caregivers' CFQ-R showed improvements in the Digestive Symptoms (T(4) - T(0) = 5.5; 95% CI = [1.5;9.4]; ES = 0.30; P < 0.05), Respiratory Symptoms (T(4) - T(0) = 7.6; 95% CI = [3.9;11.4]; ES = 0.48; P < 0.05), and Weight (T(4) - T(0) = 10.1; 95% CI = [1.6;18.6]; ES = 0.26; P < 0.05) domains., Conclusion: The introduction of dornase alfa improved the HRQL of the patients with CF during the first year of treatment., ((c) 2010 Wiley-Liss, Inc.)

This study was designed to use volumetric capnography to evaluate the breathing pattern and ventilation inhomogeneities in patients with chronic sputum production and bronchiectasis and to correlate the phase 3 slope of the capnographic curve to spirometric measurements. Twenty-four patients with cystic fibrosis (CF) and 21 patients with noncystic fibrosis idiopathic bronchiectasis (BC) were serially enrolled. The diagnosis of cystic fibrosis was based on the finding of at least two abnormal sweat chloride concentrations (iontophoresis sweat test). The diagnosis of bronchiectasis was made when the patient had a complaint of chronic sputum production and compatible findings at high-resolution computed tomography (HRCT) scan of the thorax. Spirometric tests and volumetric capnography were performed. The 114 subjects of the control group for capnographic variables were nonsmoker volunteers, who had no respiratory symptoms whatsoever and no past or present history of lung disease. Compared with controls, patients in CF group had lower SpO(2) (P < 0.0001), higher respiratory rates (RR) (P < 0.0001), smaller expiratory volumes normalized for weight (V(E)/kg) (P < 0.028), smaller expiratory times (Te) (P < 0.0001), and greater phase 3 Slopes normalized for tidal volume (P3Slp/V(E)) (P < 0.0001). Compared with controls, patients in the BC group had lower SpO(2) (P < 0.0001), higher RR (P < 0.004), smaller V(E)/kg (P < 0.04), smaller Te (P < 0.007), greater P3Slp/V(E) (P < 0.0001), and smaller VCO(2) (P < 0.0002). The pooled data from the two patient groups compared with controls showed that the patients had lower SpO(2) (P < 0.0001), higher RR (P < 0.0001), smaller V(E)/kg (P < 0.05), smaller Te (P < 0.0001), greater P3Slp/V(E) (P < 0.0001), and smaller VCO(2) (P < 0.0003). All of the capnographic and spirometric variables evaluated showed no significant differences between CF and BC patients. Spirometric data in this study reveals that the patients had obstructive defects with concomitant low vital capacities and both groups had very similar abnormalities. The capnographic variables in the patient group suggest a restrictive respiratory pattern (greater respiratory rates, smaller expiratory times and expiratory volumes, normal peak expiratory flows). Both groups of patients showed increased phase III slopes compared with controls, which probably indicates the presence of diffuse disease of small airways in both conditions leading to inhomogeneities of ventilation.

Objective: To determine the levels of alpha-1 antitrypsin (AAT) and the presence of S and Z alleles in patients with chronic respiratory symptoms., Methods: Patients with chronic cough and dyspnea were submitted to clinical evaluation, pulmonary function tests, high-resolution computed tomography, nephelometric determination of AAT and determination of S and Z alleles by polymerase chain reaction. Smoking and AAT levels were considered the dependent variables., Results: Of the 89 patients included in the study, 44 were female. The mean age was 51.3 +/- 18.2 years. The S and Z alleles were detected in 33.3% and 5.7%, respectively, and the gene frequency was 0.16 and 0.028, respectively. Two patients were SZ heterozygotes (AAT levels < 89 mg/dL). The patients were divided into groups based on AAT level: < 89 mg/dL (deficiency, no group); 90-140 mg/dL (intermediate, Group 1, n = 30); and > 141 mg/dL (normal, Group 2, n = 57). The frequency of smokers was the same in both groups, although tobacco intake was greater in Group 2. The S allele was present in 13 and 14 patients in Groups 1 and 2, respectively, whereas the Z allele was present in 2 and 1 patient in the same groups. There was no difference in the results of pulmonary function tests or in the frequency of bronchiectasis or emphysema between the two groups. Spirometric values and AAT levels were similar in smokers and nonsmokers. Bronchiectasis was more common in nonsmokers, and emphysema was more common in smokers., Conclusions: Thirty patients presented AAT levels lower than the mean values found in patients with the MM or MS genotype, and this fact could not be explained by an increased frequency of S and Z alleles.

Pulmonary thromboembolism is a common condition. Its diagnosis usually requires pulmonary scintigraphy, computed angiotomography, pulmonary arteriography and, in order to rule out other diagnoses, the measurement of D-dimer levels. Due to the fact that these diagnostic methods are not available in most Brazilian hospitals, the validation of other diagnostic techniques is of fundamental importance. We describe a case of a woman with chronic pulmonary hypertension who experienced a pulmonary thromboembolism event. Pulmonary scintigraphy, computed angiotomography and pulmonary arteriography were used in the diagnosis. The D-dimer test result was positive. Volumetric capnography was performed at admission and after treatment. The values obtained were compared with the imaging test results.

Eosinophilia-myalgia syndrome was described in 1989 in patients who presented progressive and incapacitating myalgia and eosinophilia in blood, fluids and secretions. Most patients report previous L-tryptophan intake. Respiratory manifestations are found in up to 80% of the cases, occasionally as the only manifestation. Treatment includes drug discontinuation and administration of corticosteroids. Here, we describe the case of a 61-year-old female admitted with acute respiratory failure after using L-tryptophan, hydroxytryptophan and other drugs. The patient presented eosinophilia, together with elevated eosinophil counts in the bronchoalveolar lavage and pleural effusion. After discontinuation of the drugs previously used, corticosteroids were administered, resulting in clinical and radiological improvement within just a few days.

In these case report, the results of late dead space fraction (fDlate), end-tidal alveolar dead space fraction (AVDSf), arterial-alveolar gradient CO2 [P(a-et)CO2], and slope phase 3 of spirogram of two patients who underwent thromboendarterectomy for pulmonary embolism (PE) are shown. PE was diagnosed by pulmonary scintigraphy, helical tomography, and pulmonary angiography. The calculation of fDlate, AVDSf and P(a-et)CO2 was based on volumetric capnography associated with arterial blood gas analysis. The pre-operative fDlate of the first patient was 0.16 (cutoff 0.12) and AVDSf was 0.30 (cutoff 0.15). However, the fDlate of the second patient was false-negative (0.01) but, the AVDSf was positive (0.28). Postoperative fDlate of the first patient was -0.04 and AVDSf was 0.16; for the second patient, the values were 0.07 and 0.28, respectively. The association of these capnographic variables with image exams reinforces the importance of this noninvasive diagnosis method.

The aim of this cross-sectional retrospective study was to describe clinical and functional characteristics and the microbiological profile of an adult population with cystic fibrosis. The study was performed at the Pulmonary Diseases Service of the Hospital of the Medical School of the State University of Campinas. The charts of 54 adults (27 males, 27 females) with cystic fibrosis were reviewed. Demographic, clinical, and microbiological data were collected. Clinical and functional characteristics of patients with chronic Pseudomonas infection were compared with those without it. The mean age of the patients was 41.8 years and the mean sweat chloride concentration was 106.4 mEq/L. Forty-nine percent had chronic respiratory symptoms only after 18 years of age, and 85% reported chronic production of purulent sputum. Six percent had diarrhea or fat in stools, and no patient suffered from diabetes mellitus. Pseudomonas was present in 48%, and 73% of them had the mucoid strain. Others agents found were Staphylococcus aureus (28%), Hemophylus sp. (34%), Aspergillus sp. (22%), Stenotrophomonas maltophilia (9%), Acinetobacter sp. (7%), and Burkholderia cepacia (2%). Nontuberculous mycobacteria were found in sputum from 9 patients (11%); 34 patients (67%) had obstructive ventilatory defect; and 47% had obstruction and restriction. Concerning the groups with and without Pseudomonas colonization, only the values of sweat chloride were significantly higher in chronic colonized patients. Less severe phenotypes of cystic fibrosis may be found among patients previously diagnosed as having bronchiectasis. Chronic colonization with Pseudomonas aeruginosa in this study was not associated with worse clinical status but colonized patients did show significantly higher levels of chloride in sweat.

Neuromuscular diseases affect alveolar air exchange and therefore cause chronic respiratory failure. The onset of respiratory failure can be acute, as in traumas, or progressive (slow or rapid), as in amyotrophic lateral sclerosis, muscular dystrophies, diseases of the myoneural junction, etc. Respiratory muscle impairment also affects cough efficiency and, according to the current knowledge regarding the type of treatment available in Brazil to these patients, it can be said that the high rates of morbidity and mortality in these individuals are more often related to the fact that they cough inefficiently rather than to the fact that they ventilate poorly. In this review, with the objective of presenting the options of devices available to support and substitute for natural ventilation in patients with neuromuscular diseases, we have compiled a brief history of the evolution of orthopedic braces and prostheses used to aid respiration since the end of the 19th century. In addition, we highlight the elements that are fundamental to the diagnosis of alveolar hypoventilation and of failure of the protective cough mechanism: taking of a clinical history; determination of peak cough flow; measurement of maximal inspiratory and expiratory pressures; spirometry in two positions (sitting and supine); pulse oximetry; capnography; and polysomnography. Furthermore, the threshold values available in the literature for the use of nocturnal ventilatory support and for the extension of this support through the daytime period are presented. Moreover, the maneuvers used to increase cough efficiency, as well as the proper timing of their introduction, are discussed.

Background: Pulmonary involvement is the leading cause of systemic sclerosis (SSc)-related deaths. A simple test to evaluate exercise capacity is the 6-min walk test (6MWT), and the walk distance is used as a primary outcome in clinical trials. Hemoglobin desaturation during a 6MWT is predictive of mortality in patients with primary pulmonary hypertension. Our objectives were to evaluate the walk distance and resting oxygen saturation - oxygen saturation after the 6-min period (DeltaSat) during the 6MWT in patients with SSc, and to establish correlations between the 6MWT results and other clinical variables., Methods: We analyzed 110 SSc patients. DeltaSat was defined as a fall of end-of-test saturation >or= 4%. Clinical and demographic data were collected. All the patients were submitted to chest radiographs and high-resolution CT (HRCT) and underwent pulmonary function testing and echocardiography, and the presence of autoantibodies was determined., Results: The variables associated with a walk distance < 400 m (p < 0.05) were age, dyspnea index, fibrosis on radiography, pulmonary arterial systolic pressure (PASP) >or= 30 mm Hg, and desaturation. The variables associated with DeltaSat (p < 0.05) were age, positive anti-Scl-70 autoantibody, dyspnea index, fibrosis on radiography, FVC < 80% of predicted, PASP >or= 30 mm Hg, and ground-glass or reticular opacities on HRCT. In the multivariate logistic regression analysis, three variables were significant when tested with walk distance: age, race, and dyspnea index; four variables were significant when tested with DeltaSat: age, dyspnea index, positive anti-Scl-70 autoantibody, and FVC < 80% of predicted., Conclusions: Desaturation during a 6MWT provides additional information regarding severity of disease in scleroderma patients with pulmonary manifestations.

The aim of the present study was to determine the ventilation/perfusion ratio that contributes to hypoxemia in pulmonary embolism by analyzing blood gases and volumetric capnography in a model of experimental acute pulmonary embolism. Pulmonary embolization with autologous blood clots was induced in seven pigs weighing 24.00 +/- 0.6 kg, anesthetized and mechanically ventilated. Significant changes occurred from baseline to 20 min after embolization, such as reduction in oxygen partial pressures in arterial blood (from 87.71 +/- 8.64 to 39.14 +/- 6.77 mmHg) and alveolar air (from 92.97 +/- 2.14 to 63.91 +/- 8.27 mmHg). The effective alveolar ventilation exhibited a significant reduction (from 199.62 +/- 42.01 to 84.34 +/- 44.13) consistent with the fall in alveolar gas volume that effectively participated in gas exchange. The relation between the alveolar ventilation that effectively participated in gas exchange and cardiac output (V Aeff/Q ratio) also presented a significant reduction after embolization (from 0.96 +/- 0.34 to 0.33 +/- 0.17 fraction). The carbon dioxide partial pressure increased significantly in arterial blood (from 37.51 +/- 1.71 to 60.76 +/- 6.62 mmHg), but decreased significantly in exhaled air at the end of the respiratory cycle (from 35.57 +/- 1.22 to 23.15 +/- 8.24 mmHg). Exhaled air at the end of the respiratory cycle returned to baseline values 40 min after embolism. The arterial to alveolar carbon dioxide gradient increased significantly (from 1.94 +/- 1.36 to 37.61 +/- 12.79 mmHg), as also did the calculated alveolar (from 56.38 +/- 22.47 to 178.09 +/- 37.46 mL) and physiological (from 0.37 +/- 0.05 to 0.75 +/- 0.10 fraction) dead spaces. Based on our data, we conclude that the severe arterial hypoxemia observed in this experimental model may be attributed to the reduction of the V Aeff/Q ratio. We were also able to demonstrate that V Aeff/Q progressively improves after embolization, a fact attributed to the alveolar ventilation redistribution induced by hypocapnic bronchoconstriction.

We report the case of a patient with severe dyspnea upon reclining. Lung disease, neuromuscular disorders and heart disease were ruled out. However, during the course of the investigation, bilateral diaphragmatic paresis was discovered. A key sign leading to the diagnosis was evidence of paradoxical respiration in the dorsal decubitus position. When the patient was moved from the orthostatic position to the dorsal decubitus position, oxygenation and forced vital capacity worsened. The orthostatic fluoroscopy was normal. Maximal inspiratory pressure was severely reduced. The responses to transcutaneous electric stimulation of the diaphragm were normal. However, electric stimulation of the phrenic nerve produced no response, leading to the diagnosis of bilateral diaphragmatic paresis.

Objectives: The aim of the present study was to determine the immune response profile that differentiates patients with newly diagnosed (non-treated) pulmonary tuberculosis from multidrug-resistant (MDR) ones, as well as from healthy, tuberculin positive individuals., Methods: Lymphocytes proliferative response to non-specific mitogen (PHA) and PPD were evaluated by 3H thymidine incorporation and cytokines were quantified using an ELISA assay., Results: Patients with active disease showed a diminished proliferative response to PHA and PPD, while multidrug-resistant patients showed a diminished proliferative response to PHA, but a normal response to PPD. The cytokine production of newly diagnosed patients was characterized by a diminished production of IFNgamma and normal production of transforming growth factor (TGFbeta), while MDR patients revealed a normal production of IFNgamma accompanied by an increase in TGFbeta., Conclusions: The production of significant amounts of TGFbeta in MDR patients leads to a poor immune response and may contribute to the resistance of tuberculosis patients to drugs.

This study was designed to assess the size and distribution of muscle fiber types in patients with severe chronic obstructive pulmonary disease and stable chronic hypoxemia. Brachial biceps biopsies were performed in 8 patients and 12 controls. Histochemistry was used to count and determine the cross-sectional area of the various fiber-types (1, 2a, and 2b). A significant reduction (P < 0.05) in the proportion of type 2a fibers and an increase in the proportion and cross-sectional area of type 2b fibers were seen in hypoxemic patients. These findings suggest an adaptation of the muscle fibers to a low partial pressure of oxygen in arterial blood.