ABSTRACT Objectives: To evaluate the factors associated with mortality in mechanically ventilated patients with acute respiratory distress syndrome due to COVID-19. Methods: This was a retrospective, multicenter cohort study that included 425 mechanically ventilated adult patients with COVID-19 admitted to 4 intensive care units. Clinical data comprising the SOFA score, laboratory data and mechanical characteristics of the respiratory system were collected in a standardized way immediately after the start of invasive mechanical ventilation. The risk factors for death were analyzed using Cox regression to estimate the risk ratios and their respective 95%CIs. Results: Body mass index (RR 1.17; 95%CI 1.11 - 1.20; p < 0.001), SOFA score (RR 1.39; 95%CI 1.31 - 1.49; p < 0.001) and driving pressure (RR 1.24; 95%CI 1.21 - 1.29; p < 0.001) were considered independent factors associated with mortality in mechanically ventilated patients with acute respiratory distress syndrome due to COVID-19. Respiratory system compliance (RR 0.92; 95%CI 0.90 - 0.93; p < 0.001) was associated with lower mortality. The comparative analysis of the survival curves indicated that patients with respiratory system compliance (< 30mL/cmH2O), a higher SOFA score (> 5 points) and higher driving pressure (> 14cmH2O) were more significantly associated with the outcome of death at 28 days and 60 days. Conclusion: Patients with a body mass index > 32kg/m2, respiratory system compliance < 30mL/cmH2O, driving pressure > 14cmH2O and SOFA score > 5.8 immediately after the initiation of invasive ventilatory support had worse outcomes, and independent risk factors were associated with higher mortality in this population.

RESUMO Objetivos: Avaliar os fatores associados à mortalidade em pacientes ventilados mecanicamente com síndrome o desconforto respiratório agudo por evolução da COVID-19. Métodos: Estudo de coorte retrospectiva, multicêntrica, que incluiu 425 pacientes adultos com COVID-19, ventilados mecanicamente, internados em 4 unidades de terapia intensiva. Foram coletados dados clínicos que compõem o escore SOFA, dados laboratoriais e características mecânicas do sistema respiratório, de forma padronizada, imediatamente após o início da ventilação mecânica invasiva. Os fatores de risco para óbito foram analisados por meio da regressão de Cox, para estimar as razões de risco, e seus respectivos IC95%. Resultados: Índice de massa corporal (RR de 1,17; IC95% 1,11 - 1,20; p < 0,001), escore SOFA (RR de 1,39; IC95% 1,31 - 1,49; p < 0,001) e driving pressure (RR de 1,24; IC95% 1,21 - 1,29; p < 0,001) foram considerados fatores independentes associados à mortalidade em pacientes ventilados mecanicamente com síndrome do desconforto respiratório agudo por COVID-19. Já a complacência do sistema respiratório (RR de 0,92; IC95% 0,90 - 0,93; p < 0,001) foi associada à menor mortalidade. A análise comparativa das curvas de sobrevida demonstra que pacientes com complacência do sistema respiratório (< 30mL/cmH2O), maior SOFA escore (> 5 pontos) e maior driving pressure (> 14cmH2O) apresentaram maior associação ao desfecho morte em 28 dias e 60 dias. Conclusão: Pacientes com índice de massa corporal > 32kg/m2, complacência do sistema respiratório < 30mL/cmH2O, driving pressure > 14cmH2O e SOFA escore > 5,8, imediatamente após o início da assistência ventilatória invasiva, apresentam piores desfechos no segmento, sendo fatores de risco independentes associados à maior mortalidade nessa população.

SUMMARY OBJECTIVE: The objective of this study was to evaluate the quality of life of patients with noncystic fibrosis bronchiectasis during a 1-year follow-up by using the EuroQol – 5 Dimensions – 3 Levels (EQ-5D-3L) questionnaire. METHODS: A cohort study was conducted with 100 patients with noncystic fibrosis bronchiectasis and followed up with face-to-face visits or by telephone contact every 3 months for 1 year. All patients were recruited from a single referral center for bronchiectasis. At the time of recruiting and at the end of 1 year, the EQ-5D-3L questionnaire was applied to evaluate the patients’ quality of life. Variables, such as exacerbation, emergency care, comorbidities, hemoptysis, colonization, and hospitalization, were assessed. RESULTS: Of the 100 patients, 99 completed the study and 72% were women. There were no marked limitations in the mobility and self-care domains during the follow-up. At the end of the follow-up, 32 patients were extremely anxious or depressed. The quality of life assessed by using EQ-5D-3L had an initial mean score of 0.545 and of 0.589 after 1 year, which was statistically significant (p=0.011). CONCLUSION: Patients with noncystic fibrosis bronchiectasis have a poor quality of life, and the EQ-5D-3L questionnaire may be a tool for monitoring patients with bronchiectasis.

SUMMARY OBJECTIVE: This study aims to analyze the risk factors for in-hospital mortality in a cohort of patients admitted to a newly adapted intensive care unit in a public hospital in Rio de Janeiro. METHODS: This was an observational, retrospective, and descriptive study. Data were obtained from electronic medical records. Coronavirus disease 2019 (COVID-19) was diagnosed by detecting viral ribonucleic acid using reverse transcription polymerase chain reaction. Factors associated with the risk/protection from death were determined using the odds ratio and adjusted odds ratio. RESULTS: Fifty-one patients were admitted to the hospital. The median age of the patients was 63 years, 60% were male patients, and 54% were white patients. Sixty-seven percent of the patients were diagnosed with COVID-19. Sepsis at admission increased the chance of in-hospital death by 21 times (adjusted odds ratio=21.06 [0.79–555.2]; p=0.06). The strongest risk factor for death was the development of septic shock during hospitalization (adjusted odds ratio=98.56 [2.75–352.5]; p=0.01), and one in four patients had multidrug-resistant bacteria. Mechanical ventilation, vasopressors, neuromuscular blockers, and sedatives were also the risk factors for in-hospital mortality. The in-hospital mortality rate was 41%, and the mortality rate of patients on mechanical ventilation was 60%. The diagnosis of COVID-19 was not statistically related to the adverse outcomes. CONCLUSIONS: In this cohort, the strongest risk factor for in-hospital death was the development of nosocomial septic shock. Healthcare-associated infections have a significant impact on mortality rates. Therefore, to have a better outcome, it is important to consider not only the availability of beds but also the way healthcare is delivered.

RESUMO Objetivo: A asma e a obesidade são doenças prevalentes e inter-relacionadas. Na população pediátrica, o efeito da inflamação sistêmica associada à obesidade, levando à inflamação das vias aéreas, é controverso. Nosso objetivo foi comparar padrões inflamatórios, clínicos e espirométricos entre crianças obesas e aquelas com peso normal. Métodos: Setenta e nove meninos e meninas de 6‒10 anos de idade foram selecionados e divididos em quatro grupos: asmáticos obesos, asmáticos não obesos, não asmáticos obesos e não asmáticos não obesos. Além de dados clínicos e antropométricos, todas as crianças foram submetidas a espirometria e testes cutâneos para alérgenos inalantes. Também foram coletadas amostras de sangue para dosagem de citocinas e adipocinas. Resultados: Obesos asmáticos tiveram um controle significativamente pior da asma do que os não obesos (RP 4,9; IC95% 1,1‒22,1), independentemente do sexo, atividade física e atopia. Não foram observadas diferenças nos níveis de espirometria, citocinas Th1 e Th2 e adipocinas entre os quatro grupos. Os testes cutâneos foram positivos em 81,8 e 80% dos não obesos asmáticos e obesos asmáticos, respectivamente. Conclusões: O grau de controle da asma foi significativamente menor no grupo obeso, apesar de não ter havido diferenças nos achados espirométricos. Esse resultado sugere que fatores extrapulmonares podem ser responsáveis por esse perfil sintomático. A alta positividade do teste cutâneo nos dois grupos, considerado um bom marcador de atopia, demonstrou o componente atópico como preponderante na gênese da asma, tanto em crianças com obesidade quanto naquelas com peso normal.

ABSTRACT Objective: Asthma and obesity are prevalent and interrelated diseases. In the pediatric population, the effect of systemic inflammation associated to obesity, leading to inflammation of the airways, is currently controversial. Our aim was to compare inflammatory, clinical and spirometric patterns between children with asthma and obesity and those within the normal weight status range. Methods: A total of 79 boys and girls from 6 to 10 years old were selected and divided into four groups: obese asthmatics, non-obese asthmatics, obese non-asthmatics, and non-obese non-asthmatics. In addition to collecting clinical and anthropometric data, all children underwent spirometry and skin prick tests for inhalant allergens. Blood samples for measurement of cytokines and adipokines were also collected. Results: Obese asthmatics had significantly worse control of asthma than non-obese asthmatics (OR 4.9; 95%CI 1.1‒22.1), regardless of sex, physical activity and atopy. No differences in spirometry, Th1 and Th2 cytokines and adipokines levels were observed among the four groups. The prick tests were positive in 81.8 and 80% of non-obese asthmatics and obese asthmatics, respectively. Conclusions: The degree of control of asthma was significantly lower in the obese group, regardless of the findings of no differences in spirometry. Extra-pulmonary factors could be responsible for this symptomatic profile. High positivity of skin test in both groups, which is considered a good marker of atopy, shows a preponderant atopic component in the genesis of asthma, both in children with obesity and in those within the normal weight status.

Abstract Aim: Spinal cord injury (SCI) is associated with changes in cardiac autonomic control, that can be evaluated by heart rate variability (HRV), for which the electrocardiogram (ECG) is the gold standard. However, the use of ECG is limited to laboratory environments, and new tools are needed for this purpose and that can be applied in the routine monitoring of individuals with SCI. The present study aimed to investigate the validity of the Polar V800 heart rate monitor in assessing the cardiac autonomic control of individuals with SCI. Methods: Nineteen adult men with SCI (paraplegia n = 10; 44.5 ± 8.5 years and tetraplegia n = 9; 34.4 ± 7.5 years) participated in this cross-sectional study. The participants remained in the sitting position at rest for 10 min for the acquisition of the ECG and Polar V800 signals. The last 5-min window was used to count the beat-by-beat R-R interval series and then calculate the HRV indices (linear methods in the time and frequency domains). The study subgroups were compared, and the validity of the measurements generated with a heart rate monitor was determined using the intraclass correlation coefficient (ICC2,1) and Bland-Altman graphs. Results: Agreement analyses for the R-R intervals, SDNN, rMSSD, PNN50, SD1, LF, HF, and LF: HF ratio tended to show reliability ranging from acceptable to excellent (ICC = 0.579-0.990; P = 0.043-0.001) and acceptably narrow limits of agreement within both the group with tetraplegia and the group with paraplegia. Conclusion: The Polar V800 heart rate monitor is a valid instrument for assessing HRV in individuals with paraplegia and tetraplegia.

ABSTRACT Objective To evaluate whether hormonal profile, arterial function, and physical capacity are predictors of fatigue in patients with acromegaly. Subjects and methods: This is a cross-sectional study including 23 patients. The subjects underwent a Modified Fatigue Impact Scale (MFIS) assessment; serum growth hormones (GH) and IGF-1 measurements; pulse wave analysis comprising pulse wave velocity (PWV), arterial compliance (AC), and the reflection index (IR1,2); dominant upper limb dynamometry (DYN); and the six-minute walking distance test (6MWT). Multiple linear regression models were used to identify predictors for MFIS. The coefficient of determination R2 was used to assess the quality of the models’ fit. The best model was further analyzed using a calibration plot and a limits of agreement (LOA) plot. Results The mean ± SD values for the participants’ age, MFIS, PWV, AC, IR1,2, DYN, and the distance in the 6MWT were 49.4 ± 11.2 years, 31.2 ± 18.9 score, 10.19 ± 2.34 m/s, 1.08 ± 0.46 x106 cm5/din, 85.3 ± 29.7%, 33.9 ± 9.3 kgf, and 603.0 ± 106.1 m, respectively. The best predictive model (R2 = 0.378, R2 adjusted = 0.280, standard error = 16.1, and P = 0.026) comprised the following regression equation: MFIS = 48.85 - (7.913 × IGF-I) + (1.483 × AC) - (23.281 × DYN). Conclusion Hormonal, vascular, and functional variables can predict general fatigue in patients with acromegaly.

RESUMO Objetivo avaliar os diferentes componentes da resistência do sistema respiratório e a força muscular respiratória, bem como investigar a ocorrência de limitação de fluxo expiratório (LFE) de pacientes obesos mórbidos (OM) na posição sentada. Métodos a amostra foi composta de OM (IMC ≥ 40 kg/m2) e de indivíduos não obesos (NO) com IMC entre 18 e 30 kg/m2. O protocolo foi composto de: avaliação antropométrica e da função respiratória (espirometria, pressões inspiratória (PIM) e expiratória máximas (PEM) e oscilometria de impulso). Na comparação entre os grupos, foi utilizado o teste T para amostras não pareadas. As correlações foram avaliadas pelo teste de Pearson, e o nível de significância foi de 5%. Resultados Foram avaliados 50 OM (idade 40,0 ± 10,4 anos, 1,64 ± 0,09 m, 138,8 ± 33,6 kg e 50,7 ± 8,9 kg/m2), além de 30 NO (idade 37,6 ± 11,5 anos, 1,67 ± 0,09 m, 65,2 ± 10,3 kg e 23,2 ± 22 kg/m2). Os OM apresentaram maiores valores de resistência total, central, de vias aéreas, tecidual e periférica quando comparados aos NO. Nenhum paciente apresentou LFE. A circunferência abdominal se associou com variáveis espirométricas PIM e PEM. A relação cintura-quadril se correlacionou com variáveis de mecânica respiratória, além das espirométricas PIM e PEM. Conclusões pacientes com obesidade mórbida e sem padrão espirométrico obstrutivo apresentam aumento nas resistências total, de vias aéreas, periférica e tecidual do sistema respiratório quando comparados a não obesos. Esses indivíduos, entretanto, não apresentam limitação de fluxo expiratório e redução da força muscular respiratória.

ABSTRACT Objective To evaluate the different components of the resistance of the respiratory system, respiratory muscle strength and to investigate the occurrence of expiratory flow limitation (EFL) in patients with morbid obesity (MO) when seated. Methods The sample was composed of MO (BMI≥40 kg/m2) and non-obese individuals (NO) with a BMI between 18 and 30 kg/m2. The protocol consisted of the anthropometric assessment and the following measures of respiratory function: spirometry, maximal inspiratory and expiratory pressures (MIP and MEP, respectively) and impulse oscillometry. The group comparison was performed using T-test for unpaired samples. The correlations were evaluated by the Pearson test with a significance level of 5%. Results Fifty MO (age 40±10.4 years, 1.64±0.09 m, 138.8±33.6 kg and 50.7±8.9 kg/m2), and 30 NO (age 37.6±11.5 years, 1.67±0.09 m, 65.2±10.3 kg and 23.2±22 kg/m2) were evaluated. The MO showed higher values of total, peripheral, airways, tissue and central resistance when compared to the NO. No patient showed EFL. The waist circumference was associated with spirometric variables, MIP, and MEP. The waist-to-hip ratio was correlated to respiratory mechanics and spirometric variables, MIP, and MEP. Conclusion Morbidly obese patients with no obstructive spirometric pattern show increased total, airway, peripheral, and tissue respiratory system resistance when compared to nonobese. These individuals, however, do not present with expiratory flow limitation and reduced respiratory muscles strength.

RESUMO Objetivo: Nebulizadores caseiros são usados rotineiramente no tratamento de pacientes com fibrose cística (FC). Este estudo objetiva avaliar a contaminação de nebulizadores utilizados por pacientes de FC que estão cronicamente colonizados por Pseudomonas aeruginosa e a associação da contaminação do nebulizador com a higienização, esterilização e método de secagem. Métodos: Um estudo transversal, observacional, multicêntrico foi conduzido em sete centros de referência de FC no Brasil para obter dados de registros médicos; foram feitas entrevistas estruturadas com os pacientes/cuidadores e partes de nebulizadores (máscara e copo) foram coletados para cultura microbiológica. Resultados: No geral, 77 pacientes com FC foram incluídos. A frequência da contaminação do nebulizador foi de 71,6%. Candida spp. (52,9%), Stenotrophomonas maltophilia (11,9%), P. aeruginosa não mucoide (4,8%), Staphylococcus aureus (4,8%) e complexo Burkholderia cepacia (2.4%) foram os patógenos isolados mais comuns. A frequência de higienização dos nebulizadores foi de 97,4%, e 70,3% dos pacientes relata higienização, esterilização e secagem dos aparelhos. A lavagem com água da torneira e secagem das partes no tempo, em espaço aberto, aumentou significativamente (9 a 10 vezes) a chance de contaminação dos nebulizadores. Conclusões: Apesar dos relatos de frequente higienização dos nebulizadores, os métodos de limpeza e esterilização usados eram inadequados. Uma proporção significativa de nebulizadores foi contaminada com microrganismos potencialmente patogênicos para pacientes com FC. Estes resultados apoiam a necessidade de inclusão dos pacientes/cuidadores em programas educacionais e/ou novas estratégias para fornecimento de antibióticos inalatórios.

ABSTRACT Objective: Home nebulizers are routinely used in the treatment of patients with cystic fibrosis (CF). This study aims to evaluate the contamination of nebulizers used for CF patients, that are chronically colonized by Pseudomonas aeruginosa, and the association of nebulizer contamination with cleaning, decontamination and drying practices. Methods: A cross-sectional, observational, multicenter study was conducted in seven CF reference centers in Brazil to obtain data from medical records, structured interviews with patients/caregivers were performed, and nebulizer’s parts (interface and cup) were collected for microbiological culture. Results: overall, 77 CF patients were included. The frequency of nebulizer contamination was 71.6%. Candida spp. (52.9%), Stenotrophomonas maltophilia (11.9%), non-mucoid P. aeruginosa (4.8%), Staphylococcus aureus (4.8%) and Burkholderia cepacia complex (2.4%) were the most common isolated pathogens. The frequency of nebulizers’ hygiene was 97.4%, and 70.3% of patients reported cleaning, disinfection and drying the nebulizers. The use of tap water in cleaning method and outdoor drying of the parts significantly increased (9.10 times) the chance of nebulizers’ contamination. Conclusion: Despite the high frequency hygiene of the nebulizers reported, the cleaning and disinfection methods used were often inadequate. A significant proportion of nebulizers was contaminated with potentially pathogenic microorganisms for CF patients. These findings support the need to include patients/caregivers in educational programs and / or new strategies for delivering inhaled antibiotics.

Resumo Objetivo: Descrever os achados tomográficos de pacientes com diagnóstico de infecção pulmonar pelo Mycobacterium kansasii. Materiais e Métodos: Estudo retrospectivo dos exames de tomografia computadorizada do tórax de 19 pacientes com infecção pulmonar pelo M. kansasii. Resultados: Dos 19 pacientes avaliados, 10 (52,6%) eram do sexo masculino e 9 (47,4%) eram do sexo feminino. A média de idade do grupo foi 58 anos, com variação entre 33 e 76 anos. As alterações encontradas nos exames de tomografia computadorizada foram distorção arquitetural em 17 pacientes (89,5%), opacidades reticulares e bronquiectasias em 16 (84,2%), cavidades em 14 (73,7%), nódulos centrolobulares em 13 (68,4%), pequenas consolidações em 10 (52,6%), atelectasias e grandes consolidações em 9 (47,4%), bolhas subpleurais e enfisema em 6 (31,6%) e linfonodomegalias em 1 paciente (5,3%). Conclusão: Houve predomínio de cavidades e do padrão de acometimento de pequenas e grandes vias aéreas. A doença de vias aéreas foi caracterizada por bronquiectasias e bronquiolites que se manifestaram como nódulos centrolobulares.

Abstract Objective: To describe the main tomography findings in patients diagnosed with pulmonary infection caused by Mycobacterium kansasii. Materials and Methods: Retrospective study of computed tomography scans of 19 patients with pulmonary infection by M. kansasii. Results: Of the 19 patients evaluated, 10 (52.6%) were male and 9 (47.4%) were female. The mean age of the patients was 58 years (range, 33-76 years). Computed tomography findings were as follows: architectural distortion, in 17 patients (89.5%); reticular opacities and bronchiectasis, in 16 (84.2%); cavities, in 14 (73.7%); centrilobular nodules, in 13 (68.4%); small consolidations, in 10 (52.6%); atelectasis and large consolidations, in 9 (47.4%); subpleural blebs and emphysema, in 6 (31.6%); and adenopathy, in 1 (5.3%). Conclusion: There was a predominance of cavities, as well as of involvement of the small and large airways. The airway disease was characterized by bronchiectasis and bronchiolitis presenting as centrilobular nodules.

Resumo Objetivo: Descrever e quantificar as principais alterações na tomografia computadorizada do tórax em pacientes adultos oligossintomáticos com doença falciforme e, secundariamente, avaliar o índice de acerto do radiologista quanto ao tipo de hemoglobinopatia. Materiais e Métodos: Estudo prospectivo em que 44 pacientes adultos com doença falciforme foram submetidos a tomografia computadorizada do tórax tanto em inspiração como em expiração. Foram descritos a frequência dos achados tomográficos e os graus de acometimento. Por meio da análise das alterações pulmonares e do padrão morfológico do baço, foi calculado o índice de acerto do radiologista quanto ao tipo de hemoglobinopatia. Resultados: As alterações encontradas nos exames de tomografia computadorizada, em ordem decrescente de frequência, foram: opacidades reticulares (81,8%), padrão de atenuação em mosaico (56,8%), distorção arquitetural (31,8%), cardiomegalia (25%), redução volumétrica lobar (18,2%) e aumento do calibre de ramos periféricos das artérias pulmonares (9,1%). Na maioria dos achados o grau de acometimento foi considerado leve, com até cinco segmentos pulmonares acometidos. O índice de acerto quanto ao tipo de hemoglobinopatia (grupo HbSS versus grupo não HbSS) foi 72,7%. Conclusão: Em pacientes adultos com doença falciforme os principais achados tomográficos refletem alterações fibróticas. Além do mais, a tomografia computadorizada pode ser útil na diferenciação do tipo de hemoglobinopatia.

Abstract Objective: To describe and quantify the main changes seen on computed tomography of the chest in mildly symptomatic adult patients with sickle cell disease, as well as to evaluate the radiologist accuracy in determining the type of hemoglobinopathy. Materials and Methods: A prospective study involving 44 adult patients with sickle cell disease who underwent inspiration and expiration computed tomography of the chest. The frequency of tomography findings and the extent of involvement are reported. We also calculated radiologist accuracy in determining the type of hemoglobinopathy by analyzing the pulmonary alterations and morphology of the spleen. Results: The changes found on computed tomography scans, in descending order of frequency, were as follows: fibrotic opacities (81.8%); mosaic attenuation (56.8%); architectural distortion (31.8%); cardiomegaly (25.0%); lobar volume reduction (18.2%); and increased caliber of peripheral pulmonary arteries (9.1%). For most of the findings, the involvement was considered mild, five or fewer lung segments being affected. The accuracy in determining the type of hemoglobinopathy (HbSS group versus not HbSS group) was 72.7%. Conclusion: In adult patients with sickle cell disease, the main tomography findings reflect fibrotic changes. In addition, computed tomography can be helpful in differentiating among hemoglobinopathies.

ABSTRACT Objective: To evaluate the association between clinical, pulmonary, and cardiovascular findings in patients with sickle cell disease and, secondarily, to compare these findings between sickle cell anemia patients and those with other sickle cell diseases. Methods: Fifty-nine adults were included in this cross-sectional study; 47 had sickle cell anemia, and 12 had other sickle cell diseases. All patients underwent pulmonary function tests, chest computed tomography, and echocardiography. Results: Abnormalities on computed tomography, echocardiography, and pulmonary function tests were observed in 93.5%, 75.0%; and 70.2% of patients, respectively. A higher frequency of restrictive abnormalities was observed in patients with a history of acute chest syndrome (85% vs. 21.6%; p-value < 0.0001) and among patients with increased left ventricle size (48.2% vs. 22.2%; p-value = 0.036), and a higher frequency of reduced respiratory muscle strength was observed in patients with a ground-glass pattern (33.3% vs. 4.3%; p-value = 0.016). Moreover, a higher frequency of mosaic attenuation was observed in patients with elevated tricuspid regurgitation velocity (61.1% vs. 24%; p-value = 0.014). Compared to patients with other sickle cell diseases, sickle cell anemia patients had suffered increased frequencies of acute pain episodes, and acute chest syndrome, and exhibited mosaic attenuation on computed tomography, and abnormalities on echocardiography. Conclusion: A significant interrelation between abnormalities of the pulmonary and cardiovascular systems was observed in sickle cell disease patients. Furthermore, the severity of the cardiopulmonary parameters among patients with sickle cell anemia was greater than that of patients with other sickle cell diseases.

Esclerose lateral amiotrófica é uma doença neuromuscular progressiva que resulta em fraqueza muscular, redução dos volumes pulmonares, tosse ineficaz, retenção de secreção e insuficiência respiratória. Medidas como a capacidade vital, pressão inspiratória e pressão expiratória máximas, pressão inspiratória máxima nasal, pico de fluxo de tosse e oximetria de pulso são recomendados para monitorar a função respiratória. Os pacientes devem ser acompanhados por uma equipe multidisciplinar, buscando melhorias na qualidade de vida e melhores estratégias para lidar com os sintomas respiratórios. A abordagem de cuidados respiratórios inclui técnicas de desobstrução das vias respiratórias, tosse assistida mecanicamente e ventilação mecânica não invasiva. Vacinação e suporte farmacológico também são recomendados. Até o momento, não existem provas suficientes que suportam o treinamento muscular inspiratório e a estimulação diafragmática.

Amyotrophic lateral sclerosis is a progressive neuromuscular disease, resulting in respiratory muscle weakness, reduced pulmonary volumes, ineffective cough, secretion retention, and respiratory failure. Measures as vital capacity, maximal inspiratory and expiratory pressures, sniff nasal inspiratory pressure, cough peak flow and pulse oximetry are recommended to monitor the respiratory function. The patients should be followed up by a multidisciplinary team, focused in improving the quality of life and deal with the respiratory symptoms. The respiratory care approach includes airway clearance techniques, mechanically assisted cough and noninvasive mechanical ventilation. Vaccination and respiratory pharmacological support are also recommended. To date, there is no enough evidence supporting the inspiratory muscle training and diaphragmatic pacing.

OBJECTIVE: Recent work has suggested that within-breath respiratory impedance measurements performed using the forced oscillation technique may help to noninvasively evaluate respiratory mechanics. We investigated the influence of airway obstruction on the within-breath forced oscillation technique in smokers and chronic obstructive pulmonary disease patients and evaluated the contribution of this analysis to the diagnosis of chronic obstructive pulmonary disease. METHODS: Twenty healthy individuals and 20 smokers were assessed. The study also included 74 patients with stable chronic obstructive pulmonary disease. We evaluated the mean respiratory impedance (Zm) as well as values for the inspiration (Zi) and expiration cycles (Ze) at the beginning of inspiration (Zbi) and expiration (Zbe), respectively. The peak-to-peak impedance (Zpp=Zbe-Zbi) and the respiratory cycle dependence (ΔZrs=Ze-Zi) were also analyzed. The diagnostic utility was evaluated by investigating the sensitivity, the specificity and the area under the receiver operating characteristic curve. ClinicalTrials.gov: NCT01888705. RESULTS: Airway obstruction increased the within-breath respiratory impedance parameters that were significantly correlated with the spirometric indices of airway obstruction (R=−0.65, p<0.0001). In contrast to the control subjects and the smokers, the chronic obstructive pulmonary disease patients presented significant expiratory-inspiratory differences (p<0.002). The adverse effects of moderate airway obstruction were detected based on the Zpp with an accuracy of 83%. Additionally, abnormal effects in severe and very severe patients were detected based on the Zm, Zi, Ze, Zbe, Zpp and ΔZrs with a high degree of accuracy (>90%). CONCLUSIONS: We conclude the following: (1) chronic obstructive pulmonary disease introduces higher respiratory cycle dependence, (2) this increase is proportional to airway obstruction, and (3) the within-breath forced oscillation technique may provide novel parameters that facilitate the diagnosis of respiratory abnormalities in chronic obstructive pulmonary disease.

Fundamento: Não há estudos que tenham descrito e avaliado a associação entre hemodinâmica, limitações físicas e qualidade de vida em pacientes com hipertensão pulmonar (HP) sem doença cardiovascular ou respiratória concomitante. Objetivo: Descrever o perfil hemodinâmico, a qualidade de vida e a capacidade física de pacientes com HP dos grupos I e IV e estudar a associação entre esses desfechos. Métodos: Estudo transversal em que foram incluídos pacientes com HP dos grupos clínicos I e IV e classes funcionais II e III, submetidos a avaliações hemodinâmica (cateterismo), de tolerância ao exercício e de qualidade de vida. Resultados: Foram avaliados 20 pacientes com idade média de 46,8±14,3 anos. Eles apresentaram pressão de encunhamento arterial pulmonar de 10,5±3,7 mmHg, distância percorrida no teste de caminhada de 6 minutos (DTC6M) de 463±78 m, consumo de oxigênio no pico do exercício de 12,9±4,3 mLO2.kg-1.min-1 e domínios de qualidade de vida com escores < 60%. Houve associação entre índice cardíaco (IC) e equivalente ventilatório de CO2 (r=-0,59; p<0,01), IC e equivalente ventilatório de oxigênio (r=-0,49; p<0,05), pressão atrial direita (PAD) e domínio 'estado geral de saúde' (r=-0,61; p<0,01), PAD e DTC6M (r=-0,49; p<0,05), resistência vascular pulmonar (RVP) e domínio 'capacidade funcional' (r=-0,56; p<0,01), RVP e DTC6M (r=-0,49, p<0,05) e índice de RVP e capacidade física (r=-0,51; p<0,01). Conclusão: Pacientes com HP dos grupos I e IV e classes funcionais II e III apresentam redução da capacidade física e dos componentes físico e mental de qualidade de vida. As variáveis hemodinâmicas IC, pressão arterial pulmonar diastólica, PAD, RVP e índice de RVP associam-se com a tolerância ao exercício e domínios da qualidade de vida.

Background: No studies have described and evaluated the association between hemodynamics, physical limitations and quality of life in patients with pulmonary hypertension (PH) without concomitant cardiovascular or respiratory disease. Objective: To describe the hemodynamic profile, quality of life and physical capacity of patients with PH from groups I and IV and to study the association between these outcomes. Methods: Cross-sectional study of patients with PH from clinical groups I and IV and functional classes II and III undergoing the following assessments: hemodynamics, exercise tolerance and quality of life. Results: This study assessed 20 patients with a mean age of 46.8 ± 14.3 years. They had pulmonary capillary wedge pressure of 10.5 ± 3.7 mm Hg, 6-minute walk distance test (6MWDT) of 463 ± 78 m, oxygen consumption at peak exercise of 12.9 ± 4.3 mLO2.kg-1.min-1 and scores of quality of life domains < 60%. There were associations between cardiac index (CI) and ventilatory equivalent for CO2 (r=-0.59, p <0.01), IC and ventilatory equivalent for oxygen (r=-0.49, p<0.05), right atrial pressure (RAP) and 'general health perception' domain (r=-0.61, p<0.01), RAP and 6MWTD (r=-0.49, p<0.05), pulmonary vascular resistance (PVR) and 'physical functioning' domain (r=-0.56, p<0.01), PVR and 6MWTD (r=-0.49, p<0.05) and PVR index and physical capacity (r=-0.51, p<0.01). Conclusion: Patients with PH from groups I and IV and functional classes II and III exhibit a reduction in physical capacity and in the physical and mental components of quality of life. The hemodynamic variables CI, diastolic pulmonary arterial pressure, RAP, PVR and PVR index are associated with exercise tolerance and quality of life domains.