Características demográficas, clínicas, radiológicas y de función pulmonar en pacientes con bronquiectasias / Radiological and pulmonary function clinical demographic characteristics in patients with bronchiectasias

OBJETIVOS: determinar las principales características demográficas, clínicas, radiológicas y de función pulmonar de los pacientes con bronquiectasias en la Clínica del Pulmón. MATERIAL Y MÉTODOS: estudio observacional, retrospectivo. Se revisaron las historias clínicas de 23 pacientes con diagnóstico de dilataciones bronquiales. RESULTADOS: de los 23 pacientes, con una edad media de 49,4 ± 3,87 años, 13 corresponden al sexo femenino y 10 al sexo masculino, el tiempo de evolución de la enfermedad desde el diagnóstico de la patología inicial es de 17,3 ± 2,92 años. La tuberculosis es la etiología principal en 15 pacientes (65,2%). Las manifestaciones clínicas más frecuentes son la tos y expectoración mucopurulenta por varios años en la mayoría de los pacientes, al que añadimos la disnea y hemoptisis, la auscultación pulmonar revela la presencia de crépitos en 17 pacientes (73,9%). La Tomografía de Tórax de Alta Resolución distingue dos tipos de bronquiectasias: La sacular o quística y la cilíndrica, de localización unilobar, bilobar y multilobar (difuso). La Espirometría Forzada fue indicada en 10 pacientes (43,5%) 7 mujeres y 3 varones, el Síndrome Bronquial Obstructivo fue el hallazgo más frecuente. La asociación de Tetraciclina con Metronidazol indicado en 9 pacientes (39,1%) mejoró el cuadro clínico. La fisiopatología de esta entidad clínica está sujeta a una constante actualización. CONCLUSIONES: en pacientes tosedores crónicos, las bronquiectasias deben tener prioridad diagnóstica, se trata de una patología antigua, pero de actualidad permanente.

The purpose of this document is to determine the main epidemiological and clinical characteristics of patients with bronchiectasis at the Lung Clinic. METHOD: observational, retrospective study. The medical records of 23 patients diagnosed with bronchial dilation were reviewed. RESULTS: the results of the 23 patients studied, with a mean age of 49,4 ± 3,87 years, 13 correspond to the female sex and 10 to the male sex, indicate that the time of evolution of the disease from the diagnosis of the initial pathology is: 17,3 ± 2,92 years. Tuberculosis is the main etiology in 15 patients (65,2%). The most frequent clinical manifestations were cough and mucopurulent expectoration of several years in most of the patients, to which we must add dyspnea and hemoptysis, pulmonary auscultation reveals the presence of crepitus in 17 patients (73,9%). High Resolution Chest Tomography distinguishes two types of bronchiectasis: the saccular or cystic and the cylindrical, the localization is unilobar, bilobar and multilobar (diffuse). Forced spirometry was indicated in 10 patients (43,5%), 7 women and 3 men, Chronic Obstructive Pulmonary Disease is the main diagnosis. The association of Tetracycline with Metronidazole indicated in 9 patients (39,1%) had positive results. The pathophysiology of this clinical entity is subject to constant updating. CONCLUSIONS: in chronic coughing patients, bronchiectasis must have diagnostic priority, it is an old pathology, but it is permanently current

Isolation of and risk factors for airway infection with Pseudomonas aeruginosa in patients with non-cystic fibrosis bronchiectasis / Isolamento de Pseudomonas aeruginosa e fatores de risco de infecção das vias aéreas pela bactéria em pacientes com bronquiectasias não fibrocísticas

ABSTRACT Objective: To identify microorganisms in sputum samples of patients with stable non-cystic fibrosis bronchiectasis and to determine risk factors related to the isolation of Pseudomonas aeruginosa (PA) in those patients. Methods: Consecutive patients were recruited from a tertiary hospital outpatient clinic in the city of Fortaleza, Brazil. The patients were submitted to spirometry, six-minute walk test, HRCT, and sputum collection. Data on serum fibrinogen levels, disease severity, sputum color, and history of azithromycin treatment were collected. Results: The study included 112 patients, and females predominated (68%). The mean age was 51.6 ± 17.4 years. Most patients presented with mild-to-moderate disease (83%). The mean six-minute walk distance was 468.8 ± 87.9 m. Mean FEV1 and FVC, in % of predicted values, were 60.4 ± 21.8% and 69.9 ± 18.5%, respectively. The mean serum fibrinogen level was 396.1 ± 76.3 mg/dL. PA was isolated in 47 patients, other potentially pathogenic microorganisms (PPMs) were isolated in 31 patients, and non-PPMs were isolated in 34 patients. Purulent sputum was identified in 77 patients (68%). The patients with PA, when compared with those without it, presented with more severe disease, higher serum fibrinogen levels, and lower FVC%. In addition, purulent sputum and long-term azithromycin treatment were more common in those with PA. The multivariate regression analysis showed that the independent factors associated with PA were serum fibrinogen level > 400 mg/dL (OR = 3.0; 95% CI: 1.1-7.7) and purulent sputum (OR = 4.3; 95% CI: 1.6-11.3). Conclusions: In our sample, the prevalence of PA in sputum was 42%. Sputum color and inflammatory markers were able to predict the isolation of PA, emphasizing the importance of routine sputum monitoring.

RESUMO Objetivo: Identificar microrganismos em amostras de escarro de pacientes com bronquiectasia não fibrocística estável e determinar os fatores de risco relacionados com o isolamento de Pseudomonas aeruginosa (PA) nesses pacientes. Métodos: Pacientes consecutivos foram recrutados em um ambulatório de um hospital terciário em Fortaleza (CE). Os pacientes foram submetidos a espirometria, teste de caminhada de seis minutos, TCAR e coleta de escarro. Foram coletados dados referentes ao fibrinogênio sérico, gravidade da doença, cor do escarro e histórico de tratamento com azitromicina. Resultados: O estudo incluiu 112 pacientes, com predomínio do sexo feminino (68%). A média de idade foi de 51,6 ± 17,4 anos. A maioria dos pacientes apresentou doença leve a moderada (83%). A média da distância percorrida no teste de caminhada de seis minutos foi de 468,8 ± 87,9 m. A média do VEF1 em % do previsto foi de 60,4 ± 21,8%, e a da CVF em % do previsto foi de 69,9 ± 18,5%. A média do fibrinogênio sérico foi de 396,1 ± 76,3 mg/dL. PA foi isolada em 47 pacientes; outros microrganismos potencialmente patogênicos (MPP) foram isolados em 31; não MPP foram isolados em 34. Escarro purulento foi identificado em 77 pacientes (68%). Os pacientes com PA, em comparação com aqueles sem, apresentaram doença mais grave, fibrinogênio sérico mais elevado e menor CVF%. Além disso, escarro purulento e tratamento prolongado com azitromicina foram mais comuns naqueles com PA. A análise de regressão multivariada mostrou que os fatores independentes relacionados com PA foram fibrinogênio sérico > 400 mg/dL (OR = 3,0; IC95%: 1,1-7,7) e escarro purulento (OR = 4,3; IC95%: 1,6-11,3). Conclusões: Em nossa amostra, a prevalência de PA no escarro foi de 42%. A cor do escarro e os marcadores inflamatórios foram capazes de prever o isolamento de PA, o que enfatiza a importância do monitoramento rotineiro do escarro.

Robotic thoracic surgery for inflammatory and infectious lung disease: initial experience in Brazil / Cirurgia torácica robótica para doença pulmonar inflamatória e infecciosa: experiência inicial no Brasil

ABSTRACT Objective: in Latin America, especially Brazil, the use of a robotic platform for thoracic surgery is gradually increasing in recent years. However, despite tuberculosis and inflammatory pulmonary diseases are endemic in our country, there is a lack of studies describing the results of robotic surgical treatment of bronchiectasis. This study aims to evaluate the surgical outcomes of robotic surgery for inflammatory and infective diseases by determining the extent of resection, postoperative complications, operative time, and length of hospital stay. Methods: retrospective study from a database involving patients diagnosed with bronchiectasis and undergoing robotic thoracic surgery at three hospitals in Brazil between January of 2017 and January of 2020. Results: a total of 7 patients were included. The mean age was 47 + 18.3 years (range, 18-70 years). Most patients had non-cystic fibrosis bronchiectasis (n=5), followed by tuberculosis bronchiectasis (n=1) and lung abscess (n=1). The performed surgeries were lobectomy (n=3), anatomic segmentectomy (n=3), and bilobectomy (n=1). The median console time was 147 minutes (range 61-288 min.) and there was no need for conversion to open thoracotomy. There were no major complications. Postoperative complications occurred in one patient and it was a case of constipation with the need for an intestinal lavage. The median for chest tube time and hospital stay, in days, was 1 (range, 1-6 days) and 5 (range, 2-14 days) respectively. Conclusions: robotic thoracic surgery for inflammatory and infective diseases is a feasible and safe procedure, with a low risk of complications and morbidity.

RESUMO Objetivo: na América Latina, especialmente no Brasil, a adoção da plataforma robótica para cirurgia torácica está aumentando gradativamente nos últimos anos. No entanto, apesar da tuberculose e doenças pulmonares inflamatórias serem endêmicas em nosso país, faltam estudos que descrevam os resultados do tratamento cirúrgico robótico das bronquiectasias. Este estudo tem como objetivo avaliar os resultados cirúrgicos da cirurgia robótica para doenças inflamatórias e infecciosas, determinando a extensão da ressecção, complicações pós-operatórias, tempo operatório e tempo de internação hospitalar. Métodos: estudo retrospectivo a partir de um banco de dados envolvendo pacientes com diagnóstico de bronquiectasia e submetidos à cirurgia torácica robótica em três hospitais brasileiros entre janeiro de 2017 e janeiro de 2020. Resultados: foram incluídos 7 pacientes. A média de idade foi 47 + 18,3 anos (variação, 18-70 anos). A maioria dos pacientes apresentou bronquiectasia não fibrose cística (n=5), seguida de bronquiectasia tuberculosa (n=1) e abscesso pulmonar (n=1). As cirurgias realizadas foram lobectomia (n=3), segmentectomia anatômica (n=3) e bilobectomia (n=1). O tempo médio do console foi de 147 minutos (variação de 61-288 min.) e não houve necessidade de conversão para toracotomia. Complicação pós-operatória ocorreu em um paciente, tratando-se de obstipação com necessidade de lavagem intestinal. A mediana do tempo de drenagem torácica e internação hospitalar, em dias, foi de 1 (variação, 1-6 dias) e 5 (variação, 2-14 dias), respectivamente. Conclusões: a cirurgia torácica robótica para doenças inflamatórias e infecciosas é um procedimento viável e seguro, com baixo risco de complicações e morbidade.

Risk factors for bronchiectasis in patients with chronic obstructive pulmonary disease: a systematic review and meta-analysis

The risk factors of bronchiectasis in patients with chronic obstructive pulmonary disease have not yet been established. This systematic review and meta-analysis aimed to investigate and identify potential risk factors for patients with chronic obstructive pulmonary disease accompanied by bronchiectasis. We reviewed eight electronic journal databases from their inception to November 2019 for observational studies with no language restrictions. The Newcastle-Ottawa Scale was applied to evaluate the quality of the literature. Binary variables were pooled using odds ratios and continuous variables using the standardized mean difference with 95% confidence intervals. The confidence of evidence was assessed according to the grading of the recommendations assessment, development, and evaluation method. Eight case-control studies met the inclusion criteria. Tuberculosis history, smoking history, hospitalization stays, admissions in the past year, and duration of symptoms were considered risk factors. In addition, the ratio between the forced expiratory volume in 1s and forced vital capacity, the percentage of forced expiratory volume in 1s, the forced expiratory volume in 1s as a percentage of the predicted value, purulent sputum, purulent mucus sputum, positive sputum culture, Pseudomonas aeruginosa infection, arterial oxygen pressure, daily dyspnea, C-reactive protein, leukocytes, and the percentage of neutrophils were found to be closely related to bronchiectasis. However, these were not considered risk factors. The evidence of all outcomes was judged as "low" or "very low." Additional prospective studies are required to elucidate the underlying risk factors and identify effective preventive interventions.

Bronquiectasias no relacionadas con fibrosis quística en niños: guías de diagnóstico, seguimiento y tratamiento / Bronchiectasis not related to cystic fibrosis in children: Guidelines for diagnosis, monitoring and treatment

Las bronquiectasias se encuentran dentro del espectro de enfermedad pulmonar crónica caracterizada por la dilatación bronquial progresiva y, a menudo, irreversible, causada por cambios estructurales en la pared bronquial e inflamación crónica de las vías respiratorias. El síntoma cardinal es la tos crónica persistente húmeda y productiva, que debe alertar para realizar una intervención oportuna e interrumpir el ciclo de inflamación, infección y daño de la vía aérea. Un diagnóstico precoz a través de la tomografía axial computarizada de alta resolución de tórax y el monitoreo clínico facilitan la implementación de un tratamiento intensivo que reduce y minimiza el daño de la vía aérea. Si bien las acciones terapéuticas actuales para el manejo de bronquiectasias son efectivas, existen pocos estudios clínicos aleatorizados en pediatría. El objetivo del documento es proporcionar una actualización sobre el diagnóstico, seguimiento y tratamiento de las bronquiectasias no relacionadas con fibrosis quística en niños

Bronchiectasis is within the spectrum of chronic lung disease characterized by progressive and often irreversible bronchial dilation caused by structural changes in the bronchial wall and chronic inflammation of the airways. The cardinal symptom is persistent moist and productive chronic cough that should alert to timely intervention and interrupt the cycle of inflammation, infection, and airway damage. Early diagnosis through high-resolution computed tomography of the chest and clinical monitoring facilitate the implementation of intensive treatment that reduces and minimizes damage to the airway. Although current therapeutic actions for the management of bronchiectasis are effective, there are few randomized clinical trials in pediatrics. The objective of the document is to provide an update on the diagnosis, monitoring and treatment of bronchiectasis not related to cystic fibrosis in children

Bronquiectasias no fibrosis quística: desde la infancia a la adultez: enfoque diagnostico y terapéutico / Bronchiectasis no cystic fibrosis: from childhood to adult: diagnostic and therapeutic approach

Bronchiectasis is a suppurative lung disease with heterogeneous phenotypic characteristics. It is defined as abnormal dilation of the bronchi, losing the existing relationship between bronchial sizes and accompanying artery. According to their form, they can be cylindrical, varicose, saccular or cystic. According to its location, they could be diffuse or localized. The diagnosis of bronchiectasis is usually suspected in patients with chronic cough, mucopurulent bronchorrea, and recurrent respiratory infections. The etiology can be varied, being able to classify in cystic fibrosis bronchiectasis, when there is cystic fibrosis transmembrane regulator (CFTR) gene mutation and not cystic fibrosis, being post infectious the most frequent. Its relationship with childhood is unknown. Severe respiratory infections can predispose in a susceptible subject the so-called theory of the "vicious circle" and the development of these. Persistent bacterial bronchitis in children has been described as a probable cause of not cystic fibrosis bronchiectasis in adults. The treatment is based on the management of symptoms and the prevention of exacerbations. The evidence is poor and many treatments are extrapolated from cystic fibrosis bronchiectasis. We are going to describe the diagnostic and therapeutic approach of non-cystic fibrosis bronchiectasis in adults.

La bronquiectasia es una enfermedad pulmonar supurativa con características fenotípicas heterogéneas. Se define como la dilatación anormal de los bronquios, perdiendo la relación existente entre tamaño bronquial y arteria que acompaña. Según su forma, pueden ser clasificadas en cilíndricas, varicosas, saculares o quísticas y según su etiología presentarse de forma difusa o localizada. El diagnóstico de bronquiectasias se sospecha generalmente en pacientes con tos crónica, broncorrea mucosa, mucupurulenta e infecciones respiratorias recurrentes. La etiología es variada, pudiendo clasificarse en bronquiectasias fibrosis quística, aquellas que se encuentran en el contexto de la mutación del gen regulador transmembrana de fibrosis quística (CFTR) y no fibrosis quística, de etiologías diversas, siendo post infecciosas la gran mayoría. No se conoce con certeza su relación con la infancia, es sabido que infecciones respiratorias severas pueden predisponer en un sujeto susceptible, a la llamada teoría del "circulo vicioso" y el desarrollo de estas. La bronquitis bacteriana persistente en niños se ha descrito como una causa probable del desarrollo de bronquiectasias no fibrosis quística en adultos. El tratamiento se basa en el manejo de los síntomas y la prevención de las exacerbaciones. La evidencia es escasa y la mayoría de las terapias se han investigado en las bronquiectasias tipo fibrosis quística. En este trabajo se explicará el enfrentamiento diagnóstico y terapéutico de los adultos portadores de bronquiectasias no fibrosis quística.

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.

Infección crónica por Pseudomonas aeruginosa en pacientes con bronquiectasias no fibrosis quística / Chronic infection by Pseudomonas aeruginosa in patients with non-cystic fibrosis bronchiectasias

Resumen Introducción: La infección crónica por Pseudomonas aeruginosa (PA) es frecuente en pacientes con bronquiectasias (BQ) y representa un quiebre en la historia natural de la enfermedad, asociándose a mal pronóstico y mayor severidad. Objetivo: Caracterizar la población portadora de BQ no fibrosis quística (BQ no-FQ) del Instituto Nacional del Tórax (INT) infectados crónicamente con PA comparándolos con pacientes que mantienen cultivos de expectoración negativos para este germen. Metodología: Revisión retrospectiva de fichas clínicas de pacientes portadores de BQ del INT entre julio de 2007 y abril de 2017. Se caracterizó la población y se comparó score de FACED y otros índices de gravedad. Resultados: El promedio de edad fue de 55 ± 17,3 años, 81% de los pacientes fue de género femenino. De acuerdo a aislamiento de Pseudomonas en cultivo esputo se clasificaron como infectados crónicamente (BQ con PA; n = 61) y no infectados con PA (BQ sin PA; n = 59). No hubo diferencias entre los grupos en edad y sexo. El VEF1 fue más bajo en el grupo con PA los que tienen más hospitalizaciones. Se calculó el índice de riesgo FACED siendo mayor en los pacientes colonizados. La etiología más frecuente es la postinfecciosa, principalmente secuelas de TBC, con 30,8% de etiología no identificada. Conclusiones: Los pacientes con bronquiectasias con infección crónica por Pseudomonas aeruginosa tienen una enfermedad más severa, con VEF1 más bajo, y con mayor índice de severidad de FACED. Destaca en nuestro grupo la etiología postinfecciosa.

Introduction: Chronic airways infection with Pseudomonas aeruginosa (PA) is a common situation in patients with Bronchiectasis (BQ) and constitutes a breakdown in the natural history of the latter. Moreover, BQ is also associated with a poor prognosis and an increased severity of the disease. Objective: To describe the characteristics of the population diagnosed with non-Cystic Fibrosis Bronchiectasis (non-CFB) who are chronically infected with PA, and to perform a comparison with patients with negative sputum cultures. Methodology: We performed a retrospective analysis of the clinical files of patients diagnosed with non-CFB who were attended at the 'Instituto Nacional del Tórax' (Chile) between July 2007 and April 2017. The characteristics of the population were described and the FACED scores and other severity indexes were compared. Results: The average age of patients was 55 ± 17.3 years-old, and 81% of them were female. According to PA isolation in sputum culture, they were classified as "chronically infected" (non-CFB with PA, n = 61) and "not infected with PA" (non-CFB without PA, n = 59). There were no differences in age and gender between the two groups. On the other hand, FEV1 was lower in the non-CFB PA group. The calculated FACED score was higher in colonized patients. The most frequent etiology was post-infectious, mainly TB sequels, with a 30.8% unidentified etiology. Conclusions: Patients with bronchiectasis chronically infected with Pseudomonas aeruginosa show increases in the severity of the disease, with a lower FEV1 and a higher FACED score. The postinfectious etiology is highlighted in our group.

Bronquiectasias: ¿no es más una enfermedad huérfana? / Bronchiectasis: is it no longer considered an orphan disease?

Bronchiectasis, so far considered an orphan disease, currently is diagnosed with a higher frequency due to several reasons such as renewed awareness of the disease, better diagnosis including imagenology, the development of patients registries, as well as a higher number of clinical research studies. The pathological basis of bronchiectasis is widely variable. Also the clinical expression is variable, from absence of symptoms in some patients up to chronic sputum production in others. Furthermore, a group of patients often develop recurrent exacerbations. Despite the etiologies of bronchiectasis are diverse, the main etiology is previous pulmonary infection. On the other hand, bronchiectasis could also be the expression of diverse systemic diseases. Even around one quarter of patients the etiology would not be established. The development of large registries of patients has allowed the building of classifications systems with accurate prognostic criteria. Chronic infection is the most relevant issue in bronchiectasis. Infection with P. aeruginosa has been associated with poor prognosis and their eradication must be attempted always. Effective secretions drainage techniques, oral and nebulized antibiotics, as well as mucolytic therapy are the mainstay of treatment in bronchiectasis.

Las bronquiectasias, consideradas hasta un tiempo atrás una enfermedad huérfana, se diagnostican actualmente con mayor frecuencia debido a un renovado interés en esta patología, a una mejoría de técnicas de diagnóstico, existencia de mejores registros, acceso a mejores imágenes y aumento de los estudios clínicos. El sustrato anátomo-patológico es notablemente variable, al igual que la expresión clínica, que va desde la ausencia de síntomas hasta la presencia de broncorrea crónica. A su vez, un grupo de pacientes tiende a presentar exacerbaciones frecuentes. Las etiologías de las bronquiectasias son múltiples, siendo la más frecuente la existencia de infecciones pulmonares previas. También pueden formar parte de enfermedades crónicas sistémicas. Sin embargo, en 25% de los casos no es posible identificar la etiología. El desarrollo de registros de pacientes ha permitido construir modelos de clasificación de gravedad, lo que hace posible establecer criterios pronósticos. La infección crónica es un hecho frecuente en bronquiectasias y la presencia de P aeruginosa confiere mal pronóstico a la enfermedad. La erradicación de Pseudomonas debe ser intentada siempre en estos pacientes. El uso de técnicas de drenaje, los antibióticos orales y nebulizados y las terapias mucolíticas constituyen los pilares centrales en el manejo de la enfermedad.

Qualidade de vida em voz na doença pulmonar crônica / Quality of life and voice in chronic pulmonary disease

RESUMO: Objetivo: analisar a qualidade de vida relacionada à voz autorreferida por indivíduos com doença pulmonar crônica. Métodos: estudo transversal, exploratório, quantitativo, com informações obtidas a partir da aplicação de questionário de qualidade de vida em voz em usuários de um ambulatório de fisioterapia integrado em hospital universitário no interior do Rio Grande do Sul, no período de março a novembro de 2012. Resultados: participaram 19 sujeitos, 12 (63,20%) do sexo masculino e 7 (36,80%) do sexo feminino. Sobre a faixa etária, 14 (73,70%) eram adultos e cinco (26,30%) idosos, sendo essa diferença estatisticamente significante. Quanto à doença pulmonar crônica, dez (52,60%) tinham bronquiectasia, seis (31,60%) doença pulmonar obstrutiva crônica e três (15,80%) asma. A média do questionário Qualidade de Vida em Voz Total foi de 85,8 ± 5,8 pontos. Não houve diferença estatística entre os três domínios do questionário e as variáveis sexo, idade e diagnóstico médico. Conclusão: predomínio percentual do sexo masculino e faixa etária adulta-meia idade, sendo essa última estatisticamente significante e diagnóstico médico de bronquiectasia. A média do questionário total foi de 85,8 ± 5,8 pontos. Não foram encontradas significância estatística na comparação do domínios do questionário com as variáveis sexo, idade e doença pulmonar. Tal fato pode ser explicado pela elaboração de estratégias de comunicação como forma de minimizar os efeitos da doença pulmonar na produção vocal. Sugere-se a realização de outras pesquisas abordando o mesmo tema, porém com amostras maiores a fim de verificar a significância estatística das variáveis estudadas.

ABSTRACT: Purpose: analyzing the quality of life related to self-reported voice by individuals with Chronic Pulmonary Disease. Methods: cross, exploratory and quantitative study with information obtained from the application of Quality of Life and Voice questionnaire with users an integrated physiotherapy clinic in a university hospital in the interior of Rio Grande do Sul, in the period from March to November 2012. Results: 19 subjects participated in the study, 12 (63.20%) were males and 7 (36.80%) were females. On the age group, 14 (73.70%) were adults and five (26.30%) elderly, a statistically significant difference. As for the Chronic Pulmonary Disease, ten (52.60%) had bronchiectasis, six (31.60%) had Chronic Obstructive Pulmonary Disease and three (15.80%) asthma. The average of Quality of Life and Voice questionnaire was 85.8 ± 5.8 points. There was no statistical difference between the three areas of Quality of Life and Voice questionnaire and the genre, age and medical diagnosis. Conclusion: percentage predominance of males and adult-aged age group, the latter being statistically significant and medical diagnosis of bronchiectasis. The average of the total questionnaire was 85.8 ± 5.8 points. There was no statistical significance in the comparison in questionnaire areas with the genre, age and lung disease. This can be explained by the development of communication strategies in order to minimize the effects of the Chronic Pulmonary Disease on voice. We suggest the completion of other studies addressing the same theme, but with larger samples to check the statistical significance of the studied variables.

Exacerbaciones agudas por agentes infecciosos en pacientes con enfermedad pulmonar estructural / Acute exacerbations by infectious agents in patients with structural lung disease

La enfermedad pulmonar estructural se define como toda patología que altera la arquitectura de la vía aérea inferior y el parénquima pulmonar, lo que predispone a colonización microbiológica. Entre las enfermedades catalogadas como enfermedad pulmonar estructural se encuentran la enfermedad pulmonar obstructiva crónica, las bronquiectasias y las cavitaciones como secuelas de enfermedades necrosantes. La manifestación aguda de síntomas respiratorios bajos constituye una exacerbación que deteriora el estado basal clínico, por lo que es indispensable reconocer el papel de las infecciones como causantes de estas exacerbaciones; los agentes infecciosos más comunes son: Haemophilus influenzae, Streptococcus pneumoniae y patógenos virales en la enfermedad pulmonar obstructiva crónica y Haemophilus influenzae, Moraxella catharralis y Pseudomonas aeruginosa en las bronquiectasias. En las exacerbaciones de secuelas fibrocavitarias se encuentran los mismos gérmenes de las otras alteraciones estructurales, además de hongos y micobacterias en cerca del 40%...

The structural pulmonary disease is defined as any pathology that alters the architecture of lower airway and lung parenchyma, which predisposes to microbial colonization. Among the diseases classified as structural pulmonary disease we find chronic obstructive pulmonary disease, bronchiectasis and caverns as sequelae of necrotizing diseases. Acute manifestation of lower respiratory symptoms constitutes an exacerbation that deteriorates clinical baseline condition, so it is essential to establish the role of infections as cause of these exacerbations; the most common infectious agents are: Haemophilus influenzae, Streptococcus pneumoniae and viral pathogens in chronic obstructive pulmonary disease and Haemophilus influenzae, Moraxella catarrhalis and Pseudomonas aeruginosa in bronchiectasis. In exacerbations of fibrocavitary sequelae are the same germs than other structural alterations, besides fingi and mycobacteria in about 40%...

Inhalación masiva de amoniaco: reporte de dos casos / Massive ammonia inhalation: report of two cases

Ammonia (NH3) is an irritant and corrosive gas whose inhalation at high concentrations mainly occurs during agricultural and industrial activities, as occupational accidents. The extent and severity of the damage depends on the concentration and time of exposure to the toxic, which can cause skin, eye, respiratory and life-threatening injuries. We present two cases of patients acutely exposed to high concentrations of NH3. Both patients survived to the acute phase of the respiratory injury, but developed chronic lung derangements.

El amoniaco (NH3) es un gas irritante y corrosivo cuya inhalación aguda en altas concentraciones se produce principalmente durante accidentes laborales en el sector agrícola e industrial. La extensión y severidad del daño depende de la concentración y tiempo de exposición al tóxico, el cual puede causar lesiones a nivel cutáneo, ocular, respiratorio y riesgo vital. Presentamos dos casos de pacientes expuestos en forma aguda a NH3 en altas concentraciones. Ambos pacientes sobrevivieron a la fase aguda y evolucionaron con lesiones respiratorias crónicas.

Clinical significance in non-cystic fibrosis bronchiectasis followed in a real practice / Relevância clínica em não fi brose cística bronquiectasia seguida em uma prática real

INTRODUCTION: Bronchiectasis is a chronic disorder characterized by permanent dilation of the bronchi and bronchioles accompanied by inflammatory changes in the walls of these structures and adjacent lung parenchyma. OBJECTIVE: The aim of the present study was to perform a clinical and functional characterization of adult patients with non-cystic fibrosis bronchiectasis. METHODS: A clinical, descriptive, retrospective, case-series study was carried out involving 232 patients with non-cystic fibrosis bronchiectasis treated at a lung ambulatory between 2004 and 2012. RESULTS: The sample consisted of 232 patients (134 females; mean age: 52.9 years ± 17.7; body mass index: 23.5 kg/m² ± 4.4). The predominant symptoms were cough (91.4%), expectoration (85.8%) and dyspnea (76.3%). The majority of cases were of a non-tuberculosis etiology (64.7%). Regarding lung function, the obstructive breathing pattern was predominant (43.5%). The most common comorbidities were of a cardiovascular origin (51.0%). CONCLUSIONS: Adult patients with non-cystic fibrosis bronchiectasis (mainly post-infection or post-tuberculosis in origin) are characterized by a low educational level, excessive cough, sputum, dyspnea, muscle fatigue, an obstructive breathing pattern with frequent hypoxemia and multiple comorbidities, mainly of a cardiovascular origin. However, our patients have a low index of exacerbations and hospitalizations that can be assigned to a clinical protocol for monitoring.

INTRODUÇÃO: Bronquiectasia é uma doença crônica caracterizada pela dilatação permanente dos brônquios e bronquíolos acompanhada por alterações inflamatórias nas paredes dessas estruturas e parênquima pulmonar adjacente. OBJETIVO: O objetivo do presente estudo é realizar uma caracterização clínica e funcional de pacientes adultos com bronquiectasias e fibrose não cística. Métodos: Um estudo clínico descritivo e retrospectivo foi realizado com pacientes com bronquiectasias e fibrose não cística atendidos em um ambulatório de pulmão entre 2004 e 2012. RESULTADOS: A amostra foi composta por 232 pacientes (134 mulheres, idade média: 52,9 anos ± 17,7, índice de massa corporal: 23,5 ± 4,4 kg/m2). Os sintomas predominantes foram tosse (91,4%), expectoração (85,8%) e dispneia (76,3%). A maioria dos casos foi de etiologia não tuberculosa (64,7%). Em relação à função pulmonar, o padrão de respiração obstrutiva foi predominante (43,5%). As comorbidades mais comuns foram de origem cardiovascular (51,0%). CONCLUSÕES: pacientes adultos com bronquiectasias de fibrose não cística (principalmente pós-infecção ou pós-tuberculose de origem) são caracterizados por um baixo nível de escolaridade, tosse excessiva, expectoração, dispneia, fadiga muscular, um padrão de respiração obstrutiva com hipoxemia frequente e múltiplas comorbidades, essencialmente de origem cardiovascular. No entanto, nossos pacientes têm um baixo índice de exacerbações e hospitalizações que podem ser atribuídos a um protocolo clínico para o acompanhamento.

Enfermedad de Carrington en una paciente con antecedente de bronquiectasias pulmonares: reporte de un caso / Carrington's disease in a patient with a history of bronchiectasis: a case report

La neumonía eosinofílica crónica idiopática, también conocida como enfermedad de Carrington, es una condición rara, que hace parte de un grupo heterogéneo de enfermedades que comparten el hecho de presentar infiltrados eosinofílicos en los pulmones y eosinofilia en sangre periférica. Este artículo describe el caso de una paciente con antecedente de bronquiectasias, a quien se le documentó eosinofilia en sangre periférica y en tejido pulmonar, por medio de un lavado broncoalveolar. Se trató con esteroides orales, presentó una respuesta rápida y favorable y permite el alta, luego de doce días de hospitalización.

Idiopathic chronic eosinophilic pneumonia, also known as Carrington's disease, is a rare condition, which belongs to a heterogeneous group of diseases characterized by presenting eosinophilic infiltrates in the lungs and peripheral blood eosinophilia. This article describes the case of a female patient with bronchiectasis, in whom eosinophilia was documented in peripheral blood and lung tissue by bronchoalveolar lavage. The patient, treated with oral steroids, presented a quick and favorable response, resulting in discharge after twelve days of hospitalization.

A pneumonia eosinofílica crônica idiopática, também conhecida como doença de Carrington, é uma condição rara, que faz parte de um grupo heterogêneo de doenças que compartilham o fato de apresentar infiltrados eosinofílicos nos pulmões e eosinofilia em sangue periférico. Este artigo descreve o caso de uma paciente com antecedente de bronquiectasias, a quem se lhe documentou eosinofilia no sangue periférico e no tecido pulmonar, por meio de uma lavagem broncoalveolar. Tratou-se com esteróides orais, apresentando uma resposta rápida e favorável, permitindo o alta, depois de doze dias de hospitalização.

Airway disease: similarities and differences between asthma, COPD and bronchiectasis

Airway diseases are highly prevalent worldwide; however, the prevalence of these diseases is underestimated. Although these diseases present several common characteristics, they have different clinical outcomes. The differentiation between asthma, chronic obstructive pulmonary disease and bronchiectasis in the early stage of disease is extremely important for the adoption of appropriate therapeutic measures. However, because of the high prevalence of these diseases and the common pathophysiological pathways, some patients with different diseases may present with similar symptoms. The objective of this review is to highlight the similarities and differences between these diseases in terms of the risk factors, pathophysiology, symptoms, diagnosis and treatment.

Distância percorrida no teste de caminhada de seis minutos não se relaciona com qualidade de vida em pacientes com bronquiectasias não fibrocísticas / Six-minute walk distance is not related to quality of life in patients with non-cystic fibrosis bronchiectasis

OBJETIVO: Avaliar o desempenho físico de pacientes com bronquiectasias não fibrocísticas no teste de caminhada de seis minutos (TC6) e investigar sua associação com a qualidade de vida (QV). Secundariamente, analisar a associação entre a distância percorrida no TC6 (DTC6) com achados clínicos e espirométricos para se identificar preditores para esse desempenho. MÉTODOS: Estudo transversal envolvendo pacientes com bronquiectasias não fibrocísticas, com idade > 18 anos, pelo menos um sintoma respiratório por > 2 anos e VEF1 < 70% do previsto. Os pacientes foram submetidos a avaliação clínica, teste de função pulmonar, TC6 e avaliação da QV por Medical Outcomes Study 36-item Short-Form Health Survey (SF-36). RESULTADOS: Foram incluídos 70 pacientes (48 mulheres; média de idade = 54,5 ± 17,7 anos; média de VEF1 = 44,9 ± 14,5% do previsto. Os pacientes foram divididos em dois grupos: DTC6-menor, com desempenho menor que o limite inferior previsto (n = 23); e DTC6-norm, com desempenho normal (n = 47). Em comparação ao grupo DTC6-norm, o grupo DTC6-menor apresentou menor idade, menor idade ao diagnóstico das bronquiectasias, menor proporção de ex-fumantes, menor índice de massa corpórea (IMC), menor VEF1 em % do previsto e menor PEmáx em % do previsto. Não houve diferenças significativas nos escores do SF-36 entre os grupos. No modelo de regressão logística, menor idade e menor IMC se associaram significativamente com menor DTC6. CONCLUSÕES: Nesta amostra, uma elevada proporção de pacientes apresentou uma DTC6 menor que o esperado. A DTC6 não se relacionou com a QV. Idade e IMC se associaram a DTC6.

OBJECTIVE: To evaluate physical performance on the six-minute walk test (6MWT) in patients with non-cystic fibrosis bronchiectasis and to investigate its relationship with quality of life (QoL). To identify predictors of exercise performance, we also investigated whether six-minute walk distance (6MWD) is associated with clinical and spirometric findings. METHODS: This was a cross-sectional study involving patients with non-cystic fibrosis bronchiectasis (age, > 18 years), with at least one respiratory symptom for > 2 years and an FEV1 < 70% of predicted. Patients underwent clinical evaluation, pulmonary function tests, the 6MWT, and QoL assessment with the Medical Outcomes Study 36-item Short-Form Health Survey (SF-36). RESULTS: We included 70 patients (48 females). Mean age was 54.5 ± 17.7 years, and mean FEV1 was 44.9 ± 14.5% of predicted. The patients were divided into two groups: 6MWD-low (6MWD below the predicted lower limit; n = 23); and 6MWD-norm (normal 6MWD; n = 47). The following variables were significantly lower in the 6MWD-low group than in the 6MWD-norm group: age; age at diagnosis of bronchiectasis; proportion of former smokers; body mass index (BMI); FEV1% of predicted; and MEP% of predicted. There were no significant differences in the SF-36 scores between the groups. In the logistic regression model, lower age and lower BMI were significantly associated with lower 6MWD. CONCLUSIONS: In this sample, there was a high proportion of patients who presented a lower than expected 6MWD. Although 6MWD was not related to QoL, it was associated with age and BMI.

Impact of a pulmonary rehabilitation programme on respiratory parameters and health care utilization in patients with chronic lung diseases other than COPD

Pulmonary rehabilitation is a tool that is receiving more acceptance in chronic lung diseases. A retrospective study was made in Riyadh, Saudi Arabia, on the impact of pulmonary rehabilitation on respiratory parameters and health care utilization in a group of outpatients with chronic lung diseases other than chronic obstructive pulmonary disease. A group of 51 patients diagnosed with interstitial lung diseases, bronchiectasis, asthma and scoliosis were studied. Initial number of emergency department visits and hospital admissions and use of prednisone and antibiotics were significantly associated with adherence to the pulmonary rehabilitation programme. There was a significant improvement in functional exercise capacity as manifested on the 6-minute walking distance and distance on treadmill, bicycle and arm ergometer and significantly better utilization of health care resources [fewer emergency department and outpatient department visits] over the 12 months after completion of the programme

Pneumopatias em pacientes com imunodeficiência comum variável / Pulmonary diseases in common variable immunodeficiency

A imunodeficiência comum variável (ICV) é uma imunodeficiência primária predominantemente humoral que se caracteriza pela hipogamaglobulinemia, infecções piogênicas recorrentes e outros distúrbios imunológicos, como autoimunidade e câncer. As pneumopatias são freqüentes e estão associadas a altas taxas de morbidade e mortalidade. Cerca de 80% dos pacientes com ICV apresentam pelo menos um episódio de pneumonia prévio ao diagnóstico e cerca de 30% evoluem com doença pulmonar crônica, particularmente a bronquiectasia. A avaliação pulmonar através de espirometria e exames de imagem, predominantemente a tomografia computadorizada de tórax de alta resolução, deve ser realizada precocemente nestes pacientes. Este artigo traz uma revisão das principais pneumopatias encontradas nos pacientes com ICV.

Common variable immunodeficiency (CVID) is a primary immunodeficiency, a predominantly antibody deficiency, characterized by hypogammaglobulinemia, recurrent bacterial infections, and others immunologic disorders as autoimmunity and malignity. Pulmonary complications are common and are related with high morbidity and mortality. About 80% of CVID patients have experienced at least one episode of pneumonia before diagnosis, and about 30% have chronic lung disease, particularly the development of bronchiectasis. Screening examinations, such as pulmonary function testing and high-resolution computed tomography of the chest, must be used to evaluate pulmonary status. This is a review of the major pulmonary complications in CVID patients.

Nocardiose pulmonar em portador de doença pulmonar obstrutiva crônica e bronquiectasias / Pulmonary nocardiosis in a patient with chronic obstructive pulmonary disease and bronchiectasis

Relatamos o caso de um paciente com doença pulmonar obstrutiva crônica e bronquiectasias, em uso crônico de corticosteróides, que desenvolveu nocardiose pulmonar, sob a forma de múltiplos nódulos pulmonares escavados. Os sintomas principais foram a tosse produtiva com escarro purulento, febre e dispnéia A radiografia simples e a tomografia computadorizada do tórax mostravam nódulos em ambos os pulmões, alguns escavados. O exame direto de escarro e a cultura mostraram a presença de Nocardia spp. A paciente foi tratada com imipenem e cilastatina, com excelente resposta clínica.

We report the case of a patient with chronic obstructive pulmonary disease and bronchiectasis, chronically using corticosteroids, who acquired pulmonary nocardiosis, which presented as multiple cavitated nodules. The principal symptoms were fever, dyspnea and productive cough with purulent sputum. Chest X-ray and computed tomography of the chest revealed nodules, some of which were cavitated, in both lungs. Sputum smear microscopy and culture revealed the presence of Nocardia spp. The patient was treated with imipenem and cilastatin, which produced an excellent clinical response.