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. PAH. . (PAH) (IOS airways (R (X lungs 5‒20 520 5 20 (5‒2 Hz. Hz Hz) variables R20 R5R20 RR R5-R2 area resonance frequency. frequency frequency) (IPAH 1 FEV 0.001, 0001 , 0 001 0.001) VEF1CVF VEFCVF VEF1 CVF VEF 0.049 0049 049 2575 25 75 25-7 0006 006 0.05 005 05 individuals 538 53 8 53. 007 07 0.0 kPaLs kPa L s kPa/L/s analysis X5 0036 036 0.03 0585 585 0.585 0.00 0.687, 0687 687 -0.687 0005 0.726 0726 726 -0.72 0027 027 0.02 0.610 0610 610 -0.610) 0012 012 0.01 0.669 0669 669 -0.669 0.711 0711 711 -0.711 [R (R5-R20) 0.573, 0573 573 -0.573 0020 020 0.634 0634 634 -0.63 0010 010 0.683, 0683 683 -0.683 respectively group Z 0.041, 0041 0.041 041 0.041) 0.018, 0018 0.018 018 0.018) 0.043 0043 043 0.023. 0023 0.023 023 0.023) (PAH 5‒2 52 2 (5‒ R2 R5R2 R5-R 000 00 0.04 004 04 257 7 25- 0. 003 03 058 58 0.58 0.687 068 68 -0.68 0.72 072 72 -0.7 002 02 0.61 061 61 -0.610 01 0.66 066 66 -0.66 0.71 071 71 -0.71 (R5-R20 0.573 057 57 -0.57 0.63 063 63 -0.6 0.683 5‒ (5 R5R 0.5 0.68 06 6 0.7 -0. 0.6 -0.61 (R5-R2 0.57 -0.5 ( -0 (R5-R -

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. Introduction COVID19 COVID 19 COVID-1 hospitalassociated Objectives mortality Method SARSCoV2 SARSCoV SAR SCoV 2 SAR-SCoV- 202 2021 death Descriptive comparative performed Results 65 31.5%, 315 31.5% , 31 5 (31.5%) 33.8%, 338 33.8% 33 8 (33.8%) 12.6%. 126 12.6% . 12 6 (12.6%) 30.2% 302 30 (30.2 vs 8.6% 86 0.0001, 00001 0.0001 0 0001 ventilatorassociated ventilator VAP, (VAP 525 52 52.5 12.3% 123 3 27.3% 273 27 (27.3 7.2% 72 7 23.0% 230 23 (23.0 9.9% 99 9 81.3% 813 81 (81.3 20.9% 209 20 0.0001. 15.6%, 156 15.6% 15 (15.6%) 14.4%, 144 14.4% 14 4 (14.4%) (8.6% bacteria age RR 1.03 103 1 03 95 102 02 1.0 1.05 105 05 1.05) 3.06 306 06 159 59 1.5 5.87, 587 5.87 87 5.87) 3.1 179 79 1.7 4.82 482 82 4.82) 7.76 776 76 454 54 4.5 13.26 1326 13 26 13.26) 2.2 22 1.2 3.96, 396 3.96 96 3.96) Conclusion COVID19, 19, COVID1 COVID- SAR-SCoV 31.5 (31.5% 33.8 (33.8% 12.6 (12.6% 30.2 (30. 8.6 0000 0.000 000 52. 12.3 27.3 (27. 7.2 23.0 (23. 9.9 81.3 (81. 20.9 15.6 (15.6% 14.4 (14.4% (8.6 10 1. 3.0 58 5.8 3. 17 4.8 48 7.7 77 45 4. 13.2 132 2. 39 3.9 31. (31.5 33. (33.8 12. (12.6 30. (30 8. 0.00 00 27. (27 7. 23. (23 9. 81. (81 20. 15. (15.6 14. (14.4 (8. 5. 13. (31. (33. (12. (3 0.0 (2 (8 (15. (14. (31 (33 (12 ( 0. (15 (14 (1

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.

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.

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.

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 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.

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.

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.

Em casos de asma de início recente em que o controle clínico com tratamento habitual (corticosteroide e broncodilatador) é insatisfatório, é importante considerar outros diagnósticos, tais como insuficiência cardíaca congestiva, refluxo gastroesofágico ou outras formas de obstrução das vias aéreas. Relatamos o caso de uma paciente do sexo feminino com carcinoma mucoepidermoide da traqueia mimetizando um quadro de asma brônquica. A paciente apresentava tosse e sibilância, bem como espirometria anormal com padrão obstrutivo responsivo a broncodilatador. Após um ano, apresentou deterioração clínica e espirométrica. Nenhuma anormalidade foi encontrada no radiograma de tórax. A TC revelou lesão vegetativa, a 1 cm da carina, reduzindo a luz traqueal. A fibrobroncoscopia mostrou imagem semelhante a tumor carcinoide brônquico. O diagnóstico anatomopatológico após a ressecção cirúrgica foi carcinoma mucoepidermoide de baixo grau, sem envolvimento linfonodal. Embora a curva fluxovolume não fosse sugestiva de obstrução de vias aéreas superiores, a espirometria realizada após a cirurgia mostrou redução significativa do grau de obstrução e maior reversibilidade com broncodilatador. Não houve evidência de recidiva da doença ou retorno dos sintomas após dois anos de seguimento.

In cases of recent asthma in which clinical control with the usual treatment (corticosteroids and bronchodilator) is unsatisfactory, it is important to consider other diagnoses, such as congestive heart failure, gastroesophageal reflux or other forms of airway obstruction. We report the case of a female patient with mucoepidermoid carcinoma of the trachea mimicking asthma. The patient presented cough and wheezing, as well as abnormal spirometry results with an obstructive pattern that was responsive to bronchodilators. One year later, the patient presented clinical and spirometric worsening. The chest X-ray revealed no abnormalities. A CT scan showed a vegetative lesion obstructing the tracheal lumen and located 1 cm from the carina. Fiberoptic bronchoscopy showed a finding similar to a bronchial carcinoid tumor. The anatomopathological diagnosis made after surgical resection was low-grade mucoepidermoid carcinoma, without lymph node involvement. Although the flow-volume curve was not suggestive of upper airway obstruction, the spirometry performed after the surgery showed a significant reduction in the degree of obstruction and greater reversibility after bronchodilator use. There was no evidence of recurrence of the disease or of the symptoms after a two-year follow-up period.

OBJETIVO: Determinar a concentração de alfa 1-antitripsina (AAT) e a prevalência dos alelos S e Z em indivíduos sintomáticos respiratórios crônicos. MÉTODOS: Pacientes com tosse crônica e dispnéia foram submetidos à avaliação clínica, espirometria, tomografia computadorizada de tórax, dosagem de AAT por nefelometria e pesquisa das mutações S e Z por reação em cadeia da polimerase. Foram consideradas como variáveis dependentes a concentração de AAT e o tabagismo. RESULTADOS: Dos 89 pacientes incluídos no estudo (44 mulheres; idade média, 51,3 ± 18,2 anos), os alelos S e Z foram detectados em 33,3% e 5,7%, respectivamente, com freqüência gênica dos alelos S e Z de 0,16 e 0,028. Dois pacientes tinham genótipo SZ (AAT < 89 mg/dL). Os pacientes foram divididos em grupos segundo a concentração de AAT: < 89 mg/dL (deficiência, nenhum grupo); 90-140 mg/dL (faixa intermediária, Grupo 1, n = 30); e > 141 mg/dL (normal, Grupo 2, n = 57). A freqüência de fumantes foi igual nos dois grupos, com carga tabágica maior no Grupo 2. O alelo S estava presente em 13 e 14 pacientes dos Grupos 1 e 2, respectivamente, enquanto que o alelo Z estava presente em 2 e 1 paciente dos mesmos grupos. Não houve diferença nos testes de função pulmonar, nem na freqüência de bronquiectasias ou enfisema entre os dois grupos. Os valores espirométricos e as concentrações de AAT foram similares entre fumantes e não-fumantes. Bronquiectasias foram mais freqüentes entre os não fumantes, e enfisema foi mais freqüente entre os fumantes. CONCLUSÕES: Trinta pacientes apresentaram níveis de AAT abaixo da média esperada para os genótipos MM e MS, e este fato não pode ser explicado por uma freqüência maior dos alelos S e Z.

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.

O tromboembolismo pulmonar é uma situação freqüente que pode ser diagnosticada pela cintilografia pulmonar, angiotomografia computadorizada, arteriografia pulmonar e, como método de exclusão, dosagem do dímero-D. Como estes exames nem sempre estão disponíveis, a validação de outros métodos diagnósticos é fundamental. Relata-se o caso de uma paciente com hipertensão pulmonar crônica, agudizada por tromboembolismo pulmonar. Confirmou-se o diagnóstico por cintilografia, angiotomografia computadorizada, arteriografia pulmonar; a dosagem do dímero-D resultou positiva. A capnografia volumétrica associada à gasometria arterial foi realizada na admissão e após o tratamento. As variáveis obtidas foram comparadas com os resultados dos exames de imagem.

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.

A síndrome da eosinofilia-mialgia foi descrita em 1989 em pacientes que apresentavam mialgia progressiva e incapacitante e eosinofilia sérica, nos líquidos e secreções. A maioria dos pacientes relatava uso prévio de L-triptofano. Sintomas respiratórios são relatados em até 80% dos casos, eventualmente como manifestação única. O tratamento inclui suspensão da droga e corticoterapia. Relatamos o caso de uma mulher de 61 anos com insuficiência respiratória aguda após uso de L-triptofano, hidroxitriptofano e outras drogas. A paciente apresentava eosinofilia no sangue, lavado broncoalveolar e derrame pleural. Após a suspensão da medicação e corticoterapia, houve melhora clínica e radiológica em poucos dias.

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.

As doenças neuromusculares prejudicam a renovação do ar alveolar e, por esta razão, produzem insuficiência respiratória crônica. A instalação da insuficiência respiratória pode acontecer de modo agudo, como nos traumas, ou ser lenta ou rapidamente progressiva, como na esclerose lateral amiotrófica, distrofias musculares, doença da placa mioneural, etc. O comprometimento da musculatura respiratória prejudica também a eficiência da tosse e, no estado atual da terapêutica disponível no Brasil para estes doentes, pode-se dizer que a morbimortalidade nestes indivíduos está mais associada ao fato de que eles tossem mal do que de que ventilam mal. Nesta revisão, uma breve compilação histórica procura mostrar a evolução das órteses e próteses respiratórias, desde o final do século XIX até agora, com o objetivo de apresentar as opções de máquinas disponíveis para o suporte e substituição da ventilação nas doenças neuromusculares. Além disso, são enfatizados os elementos fundamentais para o diagnóstico da hipoventilação alveolar e da falência do mecanismo protetor da tosse: história clínica, determinação do pico de fluxo da tosse, medida da pressão expiratória máxima e da pressão inspiratória máxima, espirometria em dois decúbitos (sentado e supino), oximetria de pulso, capnografia e polissonografia. São apresentados os valores limites disponíveis na literatura tanto para a indicação do suporte noturno da ventilação como para a extensão do suporte para o período diurno. As manobras para incremento da eficiência da tosse são aqui também discutidas, assim como o momento adequado para sua introdução.

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.

Relata-se o caso de um paciente com dispnéia intensa ao se deitar, em que foram excluídas doenças pulmonares, neuromusculares ou cardíacas, cuja investigação revelou paresia diafragmática bilateral. Um sinal chave para o diagnóstico foi a evidência de respiração paradoxal com o doente em decúbito supino. Havia piora da oxigenação e da capacidade vital forçada com a mudança da posição ortostática para supina. A fluoroscopia ortostática foi normal. A pressão inspiratória máxima estava muito reduzida. A estimulação elétrica transcutânea do diafragma foi normal, e a eletroestimulação do nervo frênico mostrou ausência de resposta, permitindo o diagnóstico de paresia bilateral do diafragma.

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.