Luísa Semedo

Background: Severe exacerbations and mortality are major outcomes in COPD, and risk factors for these events are actively searched for. Several predictors of mortality have been identified in COPD. The inspiratory capacity/total lung capacity (IC/TLC) ratio has been shown to be a strong predictor of all cause and respiratory mortality in patients with COPD. The major objectives of this study were to analyze which clinical parameters, including lung volumes, were the best predictors of the 5-year cumulative risk of hospital admissions or death and the 5-year risk of exacerbations, in stable COPD patients. Methods: This study retrospectively reviewed data from 98 stable COPD patients, consecutively recruited in 2012. Forced expiratory volume in 1 s (FEV 1 ), modified Medical Research Council dyspnea scale, exacerbation history (ExH), Global Initiative for Chronic Obstructive Lung Disease (GOLD) 2011 groups, and lung volumes were reviewed. Five years later, this population was evaluated for cumulative exacerbations, hospital admissions, and mortality. All the population, and GOLD group D separately, were analyzed. Results: The cumulative 5-year combined risk of hospital admission or death was significantly predicted by the ExH and the IC/TLC ratio. Analyzing separately group D, FEV 1 was the only predictor of this outcome. The frequency of exacerbations in the previous year was the best predictor of future cumulative 5-year risk of subsequent exacerbations, both for the total population and the GOLD D group. Conclusion: ExH and IC/TLC ratio were the best predictors of the most severe outcomes in COPD (admissions or mortality), independently of COPD severity. FEV 1 was the only predictor of the cumulative 5-year combined risk of hospital admission or death in the GOLD D group. ExH was the best predictor of 5-year cumulative future risk of exacerbations. Besides FEV 1 and ExH, the IC/TLC ratio can be a useful predictor of severe outcomes in COPD. Keywords: FEV 1 , severe outcomes, admissions, mortality, IC/TLC ratio

Alpha 1-antitrypsin deficiency is an inherited autosomal codominant disorder, which predisposes patients to lung and/or liver disease. Even though it is considered rare, it is one of the most frequent genetic disorders worldwide, albeit remaining underdiagnosed. Several organizations and societies, including the Portuguese Society of Pulmonology have been elaborating guidelines and recommendations for the diagnosis and management of alpha 1-antitrypsin deficiency. Nevertheless, some important matters are yet to be included in those, mainly due to lack of robust scientific evidence, and continue to represent a point of discussion. This article reviews some important scientific publications and expresses the perspectives of a group of Portuguese experts regarding the management of alpha 1-antitrypsin deficiency, namely in terms of the pre and neonatal diagnosis, the impact of the COVID-19 pandemic, the validity of replacement therapy in lung transplant-receiving, and finally, alternative strategies of alpha 1-antitrypsin deficiency treatment to improve the patients' quality of life.

<b>Introduction:</b> The 6-minute-walk test (6MWT) is a reproducible and commonly used test that evaluates exercise capacity in patients with severe pulmonary disease. The test results are considered predictors of survival in different pulmonary diseases. <b>Objective:</b> To evaluate the preoperative 6MWT results as predictors of postoperative survival in lung transplant recipients. <b>Methods:</b> A retrospective study of 97 consecutive, adult lung transplant recipients transplanted at our centre from 2009 to 2016. Kaplan-Meier methods and log rank test were used to determine the association between preoperative 6MWT results and post-transplant survival. <b>Results:</b> A total of 79 patients were included, with a mean age of 48 years (SD 13), 46 (58.2%) males, with the most common indication being interstitial lung disease (46.8%). Preoperatively, the median FEV1 was 1020 mL (33.8%), median 6-minute-walk distance (6MWD) was 296m (25th–75th percentiles =180-389 m) and oxygen desaturation was 12% (25th–75th percentiles =7-20%). The median survival was 66,46 months. Walking distance &lt; 180 m correlated with a lower survival (mean 42.2 vs 70.4 months, p=0.03). A pre-transplant higher desaturation (&gt;20%) was also associated with lower survival but without statistical significance (mean 55 vs 70 months, p=0.94). <b>Conclusions:</b> In our group 6MWD, but not oxygen desaturation, was significantly associated with post-transplant survival. This finding may have important implications for the assessment and stratification of individual risk/benefit ratio before proceeding to lung transplantation.

INTRODUCTION: Several studies suggest that severe asthmatic patients under oral steroids show higher risk of myopathy induced by steroids. Nevertheless, no conclusive data exist about the prolonged oral steroid therapy effects on the res- piratory muscles function. We didn't found correlation between the severity of obs- truction (evaluated by FEV1), hyperinflation (residual volu- me- RV) and the muscle pressures in both groups. Also, there were no significant correlation between the G1 PImax and oral steroid accumulated dose. CONCLUSION: We found a significant compromise of the res- piratory muscle function in the both studied groups with severe asthma. The oral steroid therapy caused no additional adverse effect in muscle respiratory function even after a prolon- ged treatment. The compromise of the muscle respiratory function foun- ded can be justified by the prolonged exposition to inhaled steroids. Key-words: Asthma, respiratory muscles, oral steroids.