Do Causes Influence Functional Aspects and Quality of Life in Patients with Nonfibrocystic Bronchiectasis?

Background: The denomination of noncystic fibrosis bronchiectasis (NCFB) includes several causes, and differences may be expected between the patient subgroups regarding age, comorbidities, and clinical and functional evolution. This study sought to identify the main causes of NCFB in a cohort of stable adult patients and to investigate whether such conditions would be different in their clinical, functional, and quality of life aspects. Methods: Between 2017 and 2019, all active patients with NCFB were prospectively evaluated searching for clinical data, past medical history, dyspnea severity grading, quality of life data, microbiological profile, and lung function (spirometry and six-minute walk test). Results: There was a female predominance; mean age was 54.7 years. Causes were identified in 82% of the patients, the most frequent being postinfections (n = 39), ciliary dyskinesia (CD) (n = 32), and chronic obstructive pulmonary disease (COPD) (n = 29). COPD patients were older, more often smokers (or former smokers) and with more comorbidities; they also had worse lung function (spirometry and oxygenation) and showed worse performance in the six-minute walk test (6MWT) (walked distance and exercise-induced hypoxemia). Considering the degree of dyspnea, in the more symptomatic group, patients had higher scores in the three domains and total score in SGRQ, besides having more exacerbations and more patients in home oxygen therapy. Conclusions: Causes most identified were postinfections, CD, and COPD. Patients with COPD are older and have worse pulmonary function and more comorbidities. The most symptomatic patients are clinically and functionally more severe, besides having worse quality of life.

Impulse oscillometry in patients with pulmonary arterial hypertension: an exploratory study

Abstract 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 FEV1 values (p < 0.001), VEF1/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 FEV1; [R5 and R20, (R5-R20)] also correlated with FEV1 (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.

Cohort study of hospitalized patients with COVID-19 at Brazilian tertiary-care hospital: Occurrence of adverse events and mortality

ABSTRACT Introduction: Patients hospitalized with COVID-19 are often submitted to invasive procedures and they are at risk for hospital-associated adverse events. Objectives: To evaluate the epidemiological and clinical aspects of patients hospitalized with COVID-19; the occurrence of adverse events and the risk factors for mortality. Method: Cohort study that included adult patients hospitalized with a diagnosis of SAR-SCoV-2 infection, at the tertiary University Hospital of UNICAMP from March 2020 to March 2021. Patients were identified through the hospital epidemiological surveillance system and followed until discharge or death. Descriptive, comparative, and logistic regression analysis was performed. Results: 650 adult patients were included. The main adverse events identified were nosocomial infections (31.5%), renal failure (33.8%), thromboembolic and vascular events (12.6%). Mortality was higher among those with bloodstream infections (30.2% vs. 8.6%; p < 0.0001), ventilator-associated pneumonia (VAP, 52.5% vs. 12.3%; p < 0.0001), catheter associated urinary infection (27.3% vs. 7.2%; p < 0.0001); thromboembolic and vascular events (23.0% vs. 9.9%; p < 0.0001) and renal failure (81.3% vs. 20.9%; p < 0.0001). Klebsiella pneumoniae (15.6%), Pseudomonas aeruginosa (14.4%), Enterococcus faecalis (8.6%) were the most isolated bacteria. Logistic regression analysis identified age, (RR = 1.03; 95% CI 1.02 to 1.05); ICU admission (RR = 3.06; 95% CI 1.59 to 5.87), vasoactive drug use (RR = 3.1; 95% CI 1.79 to 4.82); renal failure (RR = 7.76; 95% CI 4.54 to 13.26); and VAP (RR = 2.2; 95% CI 1.23 to 3.96), independently associated with mortality. Conclusion: adverse events have an important impact on the evolution of patients with COVID-19, reinforcing the need for optimized prevention and control measures as an essential part of care for these patients.

Bronchiectasis associated with severe COPD: Clinical, functional, microbiological and tomographic features.

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.

Lung function and stress echocardiography in pulmonary arterial hypertension: a cross-sectional study

ABSTRACT 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.

Symptom variability over the course of the day in patients with stable COPD in Brazil: a real-world observational study.

OBJECTIVE: To analyze symptoms at different times of day in patients with COPD. METHODS: This was a multicenter, cross-sectional observational study conducted at eight centers in Brazil. We evaluated morning, daytime, and nighttime symptoms in patients with stable COPD. RESULTS: We included 593 patients under regular treatment, of whom 309 (52.1%) were male and 92 (15.5%) were active smokers. The mean age was 67.7 years, and the mean FEV1 was 49.4% of the predicted value. In comparison with the patients who had mild or moderate symptoms, the 183 (30.8%) with severe symptoms were less physically active (p = 0.002), had greater airflow limitation (p < 0.001), had more outpatient exacerbations (p = 0.002) and more inpatient exacerbations (p = 0.043), as well as scoring worse on specific instruments. The most common morning and nighttime symptoms were dyspnea (in 45.2% and 33.1%, respectively), cough (in 37.5% and 33.3%, respectively), and wheezing (in 24.4% and 27.0%, respectively). The intensity of daytime symptoms correlated strongly with that of morning symptoms (r = 0.65, p < 0.001) and that of nighttime symptoms (r = 0.60, p < 0.001), as well as with the COPD Assessment Test score (r = 0.62; p < 0.001), although it showed only a weak correlation with FEV1 (r = -0.205; p < 0.001). CONCLUSIONS: Dyspnea was more common in the morning than at night. Having morning or nighttime symptoms was associated with greater daytime symptom severity. Symptom intensity was strongly associated with poor quality of life and with the frequency of exacerbations, although it was weakly associated with airflow limitation.

Brazilian consensus on non-cystic fibrosis bronchiectasis.

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.

Respiratory evaluation through volumetric capnography among grade III obese and eutrophic individuals: a comparative study

ABSTRACT 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.

Lung disease and vitamin D levels in cystic fibrosis infants and preschoolers.

INTRODUCTION: Vitamin D acts on the immune system and lung response. Patients with cystic fibrosis (CF) may be deficient in this vitamin. The aims of the study were to evaluate vitamin D levels and severity of lung disease in infants and preschoolers diagnosed with CF, and to compare them to a group of children without pancreatic insufficiency (PI). METHODS: Patients with CF up to 4 years old were included, and compared to an age-matched group of children without diagnosis of CF. CF group had medical records and High Resolution Thorax Computed Tomography (HRCCT) evaluated in order to verify the severity of lung disease. Information on demographic data, sun exposure habits, supplemental vitamin D therapy, and on the season at the time of vitamin D sampling were collected for both groups. RESULTS: This study included 45 patients in the CF group and 102 in the non-CF group, with no differences in age (P = 0.327) between them. There was no association between vitamin D levels and markers of lung disease in the CF group. The non-CF group had lower daily sun exposure (P = 0.034), and lower supplementation than the CF group (P < 0.001). Supplementation and seasonality were the determinant variables for vitamin D levels, which were lower for non-supplemented children and for assessments during fall/winter. CONCLUSION: There was no association between lung disease severity and vitamin D levels in CF group. Supplementation and seasonality were associated to higher vitamin levels.

Brazilian consensus on non-cystic fibrosis bronchiectasis / Consenso brasileiro sobre bronquiectasias não fibrocísticas

ABSTRACT 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.

RESUMO Bronquiectasias têm se mostrado uma condição cada vez mais diagnosticada com a utilização da TCAR de tórax. Na literatura, a terminologia utilizada separa as bronquiectasias entre secundárias à fibrose cística e aquelas não associadas à fibrose cística, denominadas bronquiectasias não fibrocísticas neste documento. Muitas causas podem levar ao desenvolvimento de bronquiectasias, e o paciente geralmente tem sintomas crônicos de vias aéreas, infecções recorrentes e alterações tomográficas compatíveis com a condição. Em 2010, foi publicada a primeira diretriz internacional sobre diagnóstico e tratamento das bronquiectasias não fibrocísticas. No Brasil, este é o primeiro documento de revisão com o objetivo de sistematizar o conhecimento acumulado sobre o assunto até o momento. Como para vários tópicos do tratamento não há evidências suficientes para recomendações, optou-se aqui pela construção de um documento de consenso entre especialistas. A Comissão de Infecções Respiratórias da Sociedade Brasileira de Pneumologia e Tisiologia reuniu 10 pneumologistas com expertise em bronquiectasias no Brasil para avaliar criticamente as evidências científicas e diretrizes internacionais, assim como identificar aspectos relevantes à compreensão da heterogeneidade da doença bronquiectásica e a seu manejo diagnóstico e terapêutico. Foram determinados cinco grandes tópicos (fisiopatologia; diagnóstico; monitorização do paciente estável; tratamento do paciente estável; e manejo das exacerbações). Após essa subdivisão, os tópicos foram distribuídos entre os autores, que realizaram uma revisão não sistemática da literatura, priorizando as principais publicações nas áreas específicas, incluindo artigos originais e de revisão, assim como revisões sistemáticas. Os autores revisaram e opinaram sobre todos os tópicos, formando um documento único final que foi aprovado por todos.

2018 recommendations for the management of community acquired pneumonia / Recomendações para o manejo da pneumonia adquirida na comunidade 2018

ABSTRACT Community-acquired pneumonia (CAP) is the leading cause of death worldwide. Despite the vast diversity of respiratory microbiota, Streptococcus pneumoniae remains the most prevalent pathogen among etiologic agents. Despite the significant decrease in the mortality rates for lower respiratory tract infections in recent decades, CAP ranks third as a cause of death in Brazil. Since the latest Guidelines on CAP from the Sociedade Brasileira de Pneumologia e Tisiologia (SBPT, Brazilian Thoracic Association) were published (2009), there have been major advances in the application of imaging tests, in etiologic investigation, in risk stratification at admission and prognostic score stratification, in the use of biomarkers, and in the recommendations for antibiotic therapy (and its duration) and prevention through vaccination. To review these topics, the SBPT Committee on Respiratory Infections summoned 13 members with recognized experience in CAP in Brazil who identified issues relevant to clinical practice that require updates given the publication of new epidemiological and scientific evidence. Twelve topics concerning diagnostic, prognostic, therapeutic, and preventive issues were developed. The topics were divided among the authors, who conducted a nonsystematic review of the literature, but giving priority to major publications in the specific areas, including original articles, review articles, and systematic reviews. All authors had the opportunity to review and comment on all questions, producing a single final document that was approved by consensus.

RESUMO A pneumonia adquirida na comunidade (PAC) constitui a principal causa de morte no mundo. Apesar da vasta microbiota respiratória, o Streptococcus pneumoniae permanece como a bactéria de maior prevalência dentre os agentes etiológicos. Apesar da redução significativa das taxas de mortalidade por infecções do trato respiratório inferior nas últimas décadas, a PAC ocupa o terceiro lugar como causa de mortalidade em nosso meio. Desde a última publicação das Diretrizes Brasileiras sobre PAC da Sociedade Brasileira de Pneumologia e Tisiologia (SBPT; 2009), houve importantes avanços na aplicação dos exames de imagem, na investigação etiológica, na estratificação de risco à admissão e de escores prognósticos evolutivos, no uso de biomarcadores e nas recomendações de antibioticoterapia (e sua duração) e da prevenção por vacinas. Para revisar esses tópicos, a Comissão de Infecções Respiratórias da SBPT reuniu 13 membros com reconhecida experiência em PAC no Brasil que identificaram aspectos relevantes à prática clínica que demandam atualizações frente às novas evidências epidemiológicas e científicas publicadas. Foram determinados doze tópicos envolvendo aspectos diagnósticos, prognósticos, terapêuticos e preventivos. Os tópicos foram divididos entre os autores, que realizaram uma revisão de forma não sistemática da literatura, porém priorizando as principais publicações nas áreas específicas, incluindo artigos originais, artigos de revisão e revisões sistemáticas. Todos os autores tiveram a oportunidade de revisar e opinar sobre todas as questões, criando um documento único final que foi aprovado por consenso.

Latin America validation of FACED score in patients with bronchiectasis: an analysis of six cohorts.

BACKGROUND: The FACED score is an easy-to-use multidimensional grading system that has demonstrated an excellent prognostic value for mortality in patients with bronchiectasis. A Spanish group developed the score but no multicenter international validation has yet been published. METHODS: Retrospective and multicenter study conducted in six historical cohorts of patients from Latin America including 651 patients with bronchiectasis. Clinical, microbiological, functional, and radiological variables were collected, following the same criteria used in the original FACED score study. The vital status of all patients was determined in the fifth year of follow-up. The area under ROC curve (AUC-ROC) was used to calculate the predictive power of the FACED score for all-cause and respiratory deaths and both number and severity of exacerbations. The discriminatory power to divide patients into three groups of increasing severity was also analyzed. RESULTS: Mean (SD) age of 48.2 (16), 32.9% of males. The mean FACED score was 2.35 (1.68). During the follow up, 95 patients (14.6%) died (66% from respiratory causes). The AUC ROC to predict all-cause and respiratory mortality were 0.81 (95% CI: 0.77 to 0.85) 0.84 (95% CI: 0.80 to 0.88) respectively, and 0.82 (95% CI: 078-0.87) for at least one hospitalization per year. The division into three score groups separated bronchiectasis into distinct mortality groups (mild: 3.7%; moderate: 20.7% and severe: 48.5% mortality; p < 0.001). CONCLUSIONS: The FACED score was confirmed as an excellent predictor of all-cause and respiratory mortality and severe exacerbations, as well as having excellent discriminative capacity for different degrees of severity in various bronchiectasis populations.

Quality of sweat test (ST) based on the proportion of sweat sodium (Na) and sweat chloride (Cl) as diagnostic parameter of cystic fibrosis: are we on the right way?

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.

Doença neuromuscular terminal: o cruzamento da pneumologia com a terapia intensiva / End-stage neuromuscular disease: the intersection between pulmonary and intensive care

Doenças neuromusculares evoluem com o acometimento do sistema respiratório, e a insuficiência respiratória crônica é a principal causa de mortalidade na grande maioria dos pacientes. Fraqueza da musculatura de inervação bulbar ou da musculatura respiratória podem causar tosse ineficaz e hipoventilação alveolar crônica. Disfunções de deglutição, aspiração de conteúdo gástrico, infecções de repetição e redução da complacência torácica são outras complicações pulmonares frequentes. O diagnóstico precoce, a prevenção e o tratamento destas complicações, como o uso de ventilação não invasiva e de técnicas de tosse assistida, podem melhorar significativamente a qualidade de vida e até prolongar a sobrevida destes doentes. O manejo terapêutico deve ser feito por equipe multidisciplinar, e de acordo com os desejos e preferências do paciente...

Neuromuscular diseases interfere with respiratory system function, and chronic respiratory failure is the main cause of mortality in these patients. Weakness of bulbar and respiratory muscles can impact cough efficacy and lead to chronic alveolar hypoventilation. Swallowing dysfunction, aspiration of gastric contents, recurrent respiratory infections, and reduction of thoracic compliance are all frequent events during the course of these patients. Early diagnosing, besides prevention and treatment of these events, surely ameliorate the quality of life of neuromuscular patients, and also seem to impact on survival. Therapeutic management should be multidisciplinary and must take into account the patient desires and preferences...

Volumetric capnography for the evaluation of chronic airways diseases.

BACKGROUND: 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<0.05 was considered to be a statistically significant difference. RESULTS: Concerning the NCB group (N=20) versus the control group (N=20), significant differences were found in body mass index and in several functional variables (spirometric, VC, 6MWT) with worse results observed in the NCB group. In the comparison between the COPD group (N=20) versus the NCB group, although patients with COPD had worse spirometric and 6MWT values, the capnographic variables mean phase 2 slope (Slp2), mean phase 3 slope normalized by the mean expiratory volume, or mean phase 3 slope normalized by the end-tidal CO2 concentration were similar. CONCLUSION: These findings may indicate that the gas elimination curves are not sensitive enough to monitor the severity of structural abnormalities. The role of normalized phase 3 slope may be worth exploring as a more sensitive index of small airway disease, even though it may not be equally sensitive in discriminating the severity of the alterations.

Avaliação do comprometimento respiratório em pacientes com lúpus eritematoso sistêmico com o teste de caminhada de seis minutos / Evaluation of respiratory impairment in patients with systemic lupus erythematosus with the six-minute walk test

Objetivo: Avaliar pacientes com LES estável, sem comprometimento respiratório evidente, por meio do TC6M. Casuística e métodos: Foram recrutados 45 pacientes com LES estável. Foi utilizado o protocolo ATS/ERS para TC6M, tendo sido escolhidos dois parâmetros com pontos de corte. Resultados: Quarenta e dois dos pacientes eram mulheres.A média de idade foi 39 ± 11,4 anos; a duração média da doença, 121 ± 93,1 meses; valor médio de MRC 2 ± 0; CVF média 85,9 ± 34,2%; VEF1 médio 67,5 ± 21,6%; PIM média 82 ± 58,4%; PEM média 78 ± 37,3%; frequência cardíaca média em repouso 75 ± 12,8 bpm; frequência respiratória média em repouso 19 ± 5,3 bpm; distância média no TC6M 478 ± 82 m; SpO2 média em repouso 98 ± 0,8%; queda média em SpO2 4 ± 6 pontos. Quando a população em estudo foi dividida de acordo com o valor de corte de 400 m de distância caminhada, a frequência cardíaca imediatamente antes do teste foi significativamente menor naqueles participantes que caminharam menos de 400 m (p = 0,0043), da mesma forma que o valor da escala de Borg (p = 0,0036). De acordo com a presença de saturação ≥ 4, a frequência cardíaca ao final do teste estava significativamente mais elevada naqueles participantes exibindo dessaturação (p = 0,0170); PEM (p = 0,0282) e TC6M (p = 0,0291) estavam significativamente menores e PIM revelou uma tendência para diminuir (p = 0,0504). CVF < limite inferior do normal foi achado significativamente associado com o grupo com dessaturação (p = 0,0274). Conclusão: Comparado com TC6M, a dessaturação foi o indicador mais apropriado para localizar os pacientes com os índices mais comprometidos nos testes de função respiratória. .

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. .

CFTR genotype and clinical outcomes of adult patients carried as cystic fibrosis disease.

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.

Oxygen desaturation in healthy subjects undergoing the incremental shuttle walk test / Dessaturação em indivíduos saudáveis submetidos ao incremental shuttle walk test

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. .

OBJETIVO: Determinar a probabilidade de dessaturação arterial em indivíduos saudáveis submetidos ao incremental shuttle walk test (ISWT). MÉTODOS: Foram estudados 83 indivíduos saudáveis, dos quais 55 eram homens (1 deles fumante) e 28 eram mulheres. Foram determinados VEF1 e VEF6 antes da realização do ISWT, assim como FC e SpO2 antes e depois do ISWT. RESULTADOS: As médias gerais foram as seguintes: idade, 35,05 ± 12,53 anos; índice de massa corporal, 24,30 ± 3,47 kg/m2; FC em repouso, 75,12 ± 12,48 bpm; SpO2 em repouso, 97,96 ± 1,02%; VEF1, 3,75 ± 0,81 L; VEF6, 4,45 ± 0,87 L; relação VEF1/VEF6, 0,83 ± 0,08 (sem restrição ou obstrução); distância percorrida no ISWT, 958,30 ± 146,32 m; FC pós-ISWT, 162,41 ± 18,24 bpm e SpO2 pós-ISWT, 96,27 ± 2,21% Em 11 indivíduos, houve um aumento da SpO2 após o ISWT, ao passo que em 17 houve uma queda de 4%. Não houve diferença estatística entre os grupos com e sem dessaturação após o ISWT no tocante às variáveis idade, gênero, VEF1, VEF6, VEF1/VEF6, SpO2 basal, distância percorrida no ISWT, FC e porcentagem da FC máxima. Nos indivíduos que apresentaram dessaturação, o índice de massa corporal foi maior (p = 0,01) e a SpO2 pós-ISWT foi menor (p = 0,0001). CONCLUSÕES: Indivíduos saudáveis podem apresentar dessaturação após o ISWT. O uso do ISWT para prever a presença de problemas respiratórios sutis pode ser enganador. Em indivíduos saudáveis, a dessaturação é um evento comum após o ISWT, assim ...