Jornal Brasileiro De Pneumologia最新文献

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.

Objective: Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare complication of acute pulmonary embolism, being characterized by persistent obstruction of pulmonary vessels and leading to increased pulmonary vascular resistance and right ventricular failure. Although pulmonary endarterectomy is the preferred treatment, medical therapies may offer clinical benefits in specific settings. We sought to evaluate the clinical and hemodynamic response of CTEPH patients treated with sildenafil and ambrisentan upfront combination therapy.

Methods: This was a retrospective cohort study including patients with operable and inoperable CTEPH. The patients were followed from 2019 to 2022 and were treated with sildenafil and ambrisentan as first-line therapy.

Results: Functional and hemodynamic data were analyzed at baseline and after a minimum of six months of therapy. Following treatment, there was a notable improvement in functional class, natriuretic peptide levels, and invasive hemodynamics.

Conclusions: The combined use of sildenafil and ambrisentan appears to be associated with clinical, functional, and hemodynamic improvement in patients with CTEPH.

Objective: Although communicating air pollution risks is critical for protecting public health, particularly in low- and middle-income countries (LMICs), its effectiveness remains underexplored. This study evaluated current risk communication practices in the city of Rio de Janeiro, Brazil, by assessing the associations between short-term exposure to pollutants and respiratory-related hospital admissions; the ability of the Brazilian national índice de qualidade do ar (IQAr, air quality index) to reflect health risks; and the accuracy of pollutant forecasts in comparison with monitored concentrations.

Methods: Exposure and health data for the 2014-2019 period were obtained through a research partnership with local government officials. Poisson generalized linear models were employed to determine whether IQAr values and short-term exposure to air pollutants, including nitrogen dioxide (NO2) and particulate matter (PM), were associated with daily hospital admissions for respiratory disease. Bias-corrected, forecasted daily concentrations of individual air pollutants from the Goddard Earth Observing System Composition Forecast Composition Forecast (GEOS-CF) model were employed to assess the performance of existing forecasting tools for use in risk communication.

Results: Significant associations were consistently observed between hospital admissions for respiratory disease and short-term exposures to NO2 and coarse PM, with excess risks of 5.1% (95% CI: 1.3-8.9%) and 5.6% (95% CI: 1.5-9.9%), respectively, per interquartile range increases in lag day 0-1 exposures. Values of IQAr were not significantly associated with respiratory health events, likely due to their failure to capture the health risks associated with NO2. Bias-corrected forecasts from the GEOS-CF model showed strong correlations with observed pollutant concentrations.

Conclusions: These findings indicate that adopting a health-based, multi-pollutant index, combined with improved forecasting tools, could substantially strengthen risk communication in the city of Rio de Janeiro and other LMIC settings.

Objective: Pulmonary rehabilitation programs, including exercise training, have an established role in the treatment of chronic respiratory diseases but are not routinely used in asthma. Most studies of individuals with asthma have focused on endurance training, and there is therefore limited data available on strength training. The aim of this study was to evaluate the effects that adding strength training to a program of endurance training and education has on the quality of life of such individuals.

Methods: In this single-center, parallel-group randomized controlled trial, adults with moderate-to-severe asthma admitted for in-hospital pulmonary rehabilitation between June of 2021 and October of 2022 were randomized to either a study group (SG) or a control group (CG). The SG received strength training alongside endurance training and education, whereas the CG received the same endurance training and education, along with sham mobility exercise training instead of strength training. The primary outcome was the change in the Asthma Quality of Life Questionnaire (AQLQ) score from hospital admission to discharge.

Results: A total of 61 participants were randomized, with 31 being assigned to the SG and 30 being assigned to the CG. At discharge, the AQLQ score showed significant improvement in both groups (p < 0.001 for the SG and p = 0.02 for the CG), albeit without a significant difference between the groups (p > 0.99). In contrast, peripheral muscle strength improved significantly from admission to discharge only in the SG, with a significant difference between the groups in terms of quadriceps strength (p = 0.03).

Conclusions: Adding strength training to endurance training and education does not seem to result in further improvement in the quality of life of individuals with moderate-to-severe asthma.

Objective: To establish reference values and prediction equations for the strength index (S-Index), in order to meet the growing demand for clinical application and diagnostic understanding of maximal dynamic inspiratory pressure.

Methods: This was a prospective study of 120 healthy subjects between 18 and 80 years of age. The S-Index, measured from RV to TLC after at least eight reproducible maximal maneuvers with a < 10% difference, was obtained. The MIP was also measured, and differences between S-Index and MIP values were analyzed. A multiple linear regression model estimating the S-Index value was based on clinically significant independent variables. For model cross-validation and diagnostic accuracy, we used a separate sample of COVID-19 survivors to compare the observed and predicted S-Index values.

Results: The S-Index strongly correlated with the FEV1 and FVC. However, sex, age, weight, and height retained their significance in all final models, collectively explaining 62% of the variation in the observed values. The performance of the prediction equation was satisfactory in suggesting differences between COVID-19 survivors with an MIP < 80 cmH2O and those with an MIP ≥ 80 cmH2O. For both sexes, the S-Index exhibited the potential for ruling out, rather than confirming, inspiratory muscle weakness. If below the lower limit of normal, further evaluation is important, especially in men.

Conclusions: To our knowledge, this is the first set of reference equations for the S-Index based on a healthy adult population across various age groups in Brazil. Its potential as an adjunct index in evaluating inspiratory muscle strength was also explored for the first time.