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

Extracorporeal membrane oxygenation and lung transplantation: initial experience at a single brazilian center

OBJECTIVE: To report initial experience from the use of extracorporeal membrane oxygenation (ECMO) in patients who received lung transplantation. METHODS: Retrospective study of a single tertiary center in the Brazilian state of São Paulo, a national reference in lung transplantation, based on the prospective collection of data from electronic medical records. The period analyzed extended from January 2009 (beginning of the program) until December 2018. RESULTS: A total of 75 lung transplants were performed, with ECMO used in 8 (10.7%) cases. Of the patients, 4 (50%) were female. The mean age was 46.4±14.3 years. The causes of the end-stage lung disease that led to transplantation were pulmonary arterial hypertension in 3 (37.5%) patients, bronchiectasis in 2 (25%) patients, pulmonary fibrosis in 2 (25%) patients, and pulmonary emphysema in 1 (12.5%) patient. In our series, 7 (87.5%) cases were sequential bilateral transplantations. Prioritization was necessary in 4 (50%) patients, and in 1 patient, ECMO was used as a bridge to transplantation. The ECMO route was central in 4 (50%), peripheral venovenous in 2 (25%) and peripheral venoarterial in 2 (25%) patients. The mean length of the intensive care unit (ICU) stay was 14±7.5 days and of the hospital stay was 34.1±34.2 days. The mean ECMO duration was 9.3±6.6 days with a 50% decannulation rate. Three patients were discharged (37.5%). CONCLUSION: Lung transplantation requires complex treatment, and ECMO has allowed extending the indications for transplantation and provided adjuvant support in the clinical management of these patients.

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.

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.

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.

Efeitos da ELTGOL e do Flutter® nos volumes pulmonares dinâmicos e estáticos e na remoção de secreção de pacientes com bronquiectasia / Effects of ELTGOL and Flutter VRP1® on the dynamic and static pulmonary volumes and on the secretion clearance of patients with bronchiectasis

Contextualização: Embora a fisioterapia respiratória seja considerada fundamental para o tratamento de pacientes hipersecretivos, há poucas evidências acerca de seus efeitos fisiológicos e terapêuticos em indivíduos com bronquiectasia. Objetivos: Avaliar os efeitos fisiológicos imediatos da ELTGOL e do Flutter® nos volumes pulmonares dinâmicos e estáticos em pacientes com bronquiectasia e, secundariamente, determinar o efeito dessas técnicas na remoção de secreção brônquica. Métodos: Participaram do estudo pacientes com diagnóstico clínico e radiológico de bronquiectasia. Os pacientes foram submetidos a três intervenções de forma randomizada e com um intervalo (washout) de uma semana entre elas. Inicialmente os pacientes inalaram dois jatos de 100µcg de salbutamol. Após 5 minutos de tosse iniciais e após 5 minutos de tosse que sucederam o protocolo controle e as intervenções (ELTGOL e Flutter®), os pacientes realizaram as avaliações dos volumes pulmonares dinâmicos e estáticos por meio da espirometria e pletismografia corporal. A secreção expectorada foi coletada durante as intervenções e durante a segunda série de tosse, sendo quantificada por meio de seu peso seco. Resultados: Foram avaliados dez pacientes, dois do sexo masculino e oito do sexo feminino (média de idade de 55,9±18,1 anos). Após a utilização do Flutter® e da ELTGOL, observou-se diminuição significativa do volume residual (VR), da capacidade residual funcional (CRF) e da CPT (p<0,05). Foi eliminada maior quantidade de secreção pulmonar durante a ELTGOL em comparação com o Controle e o Flutter®. Conclusão: O Flutter VRP1® e a técnica ELTGOL reduziram a hiperinsuflação pulmonar a curto prazo, porém apenas o ELTGOL aumentou a eliminação de secreção pulmonar de pacientes com bronquiectasia.

Background: Although respiratory physical therapy is considered fundamental in the treatment of hypersecretive patients, there is little evidence of its physiological and therapeutic effects in bronchiectasis patients. Objective: To evaluate the acute physiological effects of ELTGOL and Flutter VRP1® in dynamic and static lung volumes in patients with bronchiectasis and, secondarily, to study the effect of these techniques in sputum elimination. Methods: Patients with clinical and radiological diagnosis of bronchiectasis were included. Patients underwent three interventions in a randomized order and with a one-week washout interval between them. Before all interventions patients inhaled two puffs of 100 mcg of salbutamol. There was a cough period of five minutes before and after the control protocol and the interventions (ELTGOL and Flutter VRP1®). After each cough series patients underwent assessments of dynamic and static lung volumes by spirometry and plethysmography. The expectorated secretions were collected during the interventions and during the second cough series, and quantified by its dry weight. Results: We studied 10 patients, two males and eight females (mean age: 55.9±18.1 years). After using Flutter VRP1®and ELTGOL there was a significant decrease in residual volume (RV), functional residual capacity (FRC) and total lung capacity (TLC) (p<0.05). There was a higher sputum production during ELTGOL compared with Control and Flutter VRP1® (p<0.05). Conclusion: The ELTGOL and Flutter VRP1® techniques acutely reduced lung hyperinflation, but only the ELTGOL increased the removal of pulmonary secretions from patients with bronchiectasis.

Bronquiectasias en pediatría, aproximación diagnóstica y tratamiento / Bronchiectasis in pediatrics, diagnosis approach and management

Las bronquiectasias son un problema de salud en países desarrollados y en vías de desarrollo. La fibrosis quística es una causa importante, si bien fuera de ésta existen causas que convierten a las bronquiectasias no relacionadas con fibrosis quística en un diagnóstico frecuente. Su diagnóstico precoz basado en la clínica y posterior confirmación radiológica es el punto de partida para determinar la etiología e instaurar un tratamiento dirigido a la causa subyacente. Para ello es menester una evaluación ordenada y sistemática. Se presenta una revisión sobre la etiología y tratamiento de las bronquiectasias, especialmentede las no relacionadas con fibrosis quística.

Surgery for bronchiectasis: the effect of morphological types to prognosis

Although the incidence has declined over the past years in societies with high socioeconomic status, bronchiectasis is still an important health problem in our country. To review and present our cases undergoing surgery for bronchiectasis in the past 12 years and their early and late term postoperative outcomes and our experience in bronchiectasis surgery and the effect of morphological type on the prognosis. The medical records of 62 cases undergoing surgical resection for bronchiectasis in the Clinics of Thoracic and Pediatric Surgery were evaluated retrospectively. The disease was on the left in 33 cases, on the right in 26 and bilateral in three cases. The most common surgical procedure was lobectomy. Forty one patients underwent pneumonectomy, lobectomy and complete resection including bilobectomy. Twenty-one [33.87%] cases underwent incomplete resection, of whom 11 [17.74%] underwent segmentectomy and 10 [16.13%] underwent lobectomy + segmentectomy. It was found that the rate of being asymptomatic was significantly higher in patients undergoing complete resection compared to those undergoing incomplete resection. Spirometric respiratory function tests were performed to assess the relationship between morphological type and the severity of disease. All parameters of respiratory function were worse in the saccular type and FEV 1 /FVC showed a worse obstructive deterioration in the saccular type compared to the tubular type. The success rate of the procedure increases with complete resection of the involved region. The morphological type is more important than the number and extension of the involved segments in showing the disease severity

Deve-se extrapolar o tratamento de bronquiectasias em pacientes com fibrose cística para aqueles com bronquiectasias de outras etiologias? / Should the bronchiectasis treatment given to cystic fibrosis patients be extrapolated to those with bronchiectasis from other causes?

OBJETIVO: Conhecer o perfil de pacientes adultos com bronquiectasias, comparando portadores de fibrose cística (FC) com aqueles com bronquiectasias de outra etiologia, a fim de determinar se é racional extrapolar terapêuticas instituídas em fibrocísticos para aqueles com bronquiectasias de outras etiologias. MÉTODOS: Análise retrospectiva dos prontuários de 87 pacientes adultos com diagnóstico de bronquiectasia em acompanhamento em nosso serviço. Pacientes com doença secundária a infecção por tuberculose corrente ou no passado foram excluídos. Foram avaliados dados clínicos, funcionais e terapêuticos dos pacientes. RESULTADOS: Dos 87 pacientes com bronquiectasias, 38 (43,7 por cento) tinham diagnóstico confirmado de FC através de dosagem de sódio e cloro no suor ou análise genética, enquanto 49 (56,3 por cento) apresentavam a doença por outra etiologia, 34 (39,0 por cento) desses com bronquiectasia idiopática. Os pacientes com FC apresentavam média de idade ao diagnóstico mais baixa (14,2 vs. 24,2 anos; p < 0,05). A prevalência de sintomas (tosse, expectoração, hemoptise e sibilância) foi semelhante entre os grupos. A colonização por Pseudomonas aeruginosa e a por Staphylococcus aureus foram mais comuns nos pacientes com FC (82,4 vs. 29,7 por cento e 64,7 vs. 5,4 por cento, respectivamente). CONCLUSÕES: As causas e as manifestações clínicas das bronquiectasias são heterogêneas, sendo importante a identificação dessas diferenças na abordagem do paciente. Reconhecer estas diferenças é crucial para o desenvolvimento de novas estratégias para o manejo de pacientes com bronquiectasias.

OBJECTIVE: To profile the characteristics of adult patients with bronchiectasis, drawing comparisons between cystic fibrosis (CF) patients and those with bronchiectasis from other causes in order to determine whether it is rational to extrapolate the bronchiectasis treatment given to CF patients to those with bronchiectasis from other causes. METHODS: A retrospective analysis of the medical charts of 87 patients diagnosed with bronchiectasis and under follow-up treatment at our outpatient clinic. Patients who had tuberculosis (current or previous) were excluded. We evaluated the clinical, functional, and treatment data of the patients. RESULTS: Of the 87 patients with bronchiectasis, 38 (43.7 percent) had been diagnosed with CF, through determination of sweat sodium and chloride concentrations or through genetic analysis, whereas the disease was due to another etiology in 49 (56.3 percent), of whom 34 (39.0 percent) had been diagnosed with idiopathic bronchiectasis. The mean age at diagnosis was lower in the patients with CF than in those without (14.2 vs. 24.2 years; p < 0.05). The prevalence of symptoms (cough, expectoration, hemoptysis, and wheezing) was similar between the groups. Colonization by Pseudomonas aeruginosa or Staphylococcus aureus was more common in the CF patients (82.4 vs. 29.7 percent and 64.7 vs. 5.4 percent, respectively). CONCLUSIONS: The causes and clinical manifestations of bronchiectasis are heterogeneous, and it is important to identify the differences. It is crucial that these differences be recognized so that new strategies for the management of patients with bronchiectasis can be developed.

Influência da técnica de pressão expiratória positiva oscilante utilizando pressões expiratórias pré-determinadas na viscosidade e na transportabilidade do escarro em pacientes com bronquiectasia / Influence that oscillating positive expiratory pressure using predetermined expiratory pressures has on the viscosity and transportability of sputum in patients with bronchiectasis

OBJETIVO: Verificar a efetividade da técnica de pressão expiratória positiva oscilante (PEPO) utilizando pressões expiratórias pré-determinadas sobre a viscosidade e a transportabilidade do escarro em pacientes com bronquiectasia. MÉTODOS: Foram incluídos no estudo 15 pacientes estáveis com bronquiectasia (7 homens; média de idade = 53 ± 16 anos), submetidos a duas intervenções PEPO consecutivas, com 24 h de intervalo entre si, utilizando pressões expiratórias de 15 cmH2O (P15) e 25 cmH2O (P25). O protocolo consistiu de tosse voluntária; nova expectoração voluntária após 20 min, denominado tempo zero (T0); repouso de 10 min; e utilização da técnica em duas séries de 10 min (S1 e S2) de PEPO em P15 e P25, com intervalo de 10 min entre si. A viscosidade e transportabilidade do escarro foram avaliadas pela viscosimetria, velocidade relativa de transporte no palato de rã, deslocamento em máquina simuladora de tosse e ângulo de adesão. As amostras de escarro foram coletadas em T0, após S1 e após S2. Testes estatísticos específicos foram aplicados de acordo com a distribuição dos dados. RESULTADOS: Houve diminuição significante da viscosidade do escarro após S1 em P15 e após S2 em P25. Não houve diferenças significantes entre todas as amostras para a transportabilidade. CONCLUSÕES: Houve diminuição da viscosidade do escarro quando a PEPO foi realizada em P15 e P25, o que sugere que não seja necessário gerar alta pressão expiratória para obter o resultado desejado.

OBJECTIVE: To determine the effectiveness of oscillating positive expiratory pressure (OPEP) using predetermined expiratory pressures on the viscosity and transportability of sputum in patients with bronchiectasis. METHODS: The study involved 15 stable patients with bronchiectasis (7 males; mean age = 53 ± 16 years), submitted to two consecutive OPEP interventions, with a 24-h interval between the two, using positive expiratory pressures set at 15 cmH2O (P15) and 25 cmH2O (P25). The protocol consisted of a voluntary cough; another voluntary cough 20 min later, designated time zero (T0); a 10-min rest period; and two 10-min series (S1 and S2, using OPEP at P15 and P25 in both), with a 10-min interval between the two. The viscosity and transportability of sputum were evaluated by viscometry, relative transport velocity on frog palate, transport in a simulated cough machine and contact angle. Sputum samples were collected at T0, after S1 and after S2. Specific statistical tests were performed depending on the type of data distribution. RESULTS: In comparison with the values obtained at T0, sputum viscosity decreased significantly after S1 at P15 and after S2 at P25. There were no significant differences among all of the samples in terms of transportability. CONCLUSIONS: The fact that sputum viscosity decreased whether OPEP was performed at P15 or at P25 suggests that there is no need to generate high expiratory pressure to achieve the desired result.

[Aetiologies and outcome of bronchiectasis in children: a study of 41 patients]

Bronchiectasis remains an important cause of chronic suppurative lung disease in the developing world. This study is to describe the epidemiological characteristics, clinical features, underlying aetiologies and Outcome of bronchiectasis in the paediatric hospital of Tunis. A retrospective study of 41 children with bronchiectasis was conducted between January 1994 and December 2006. Diagnosis was made in patients with clinical suspicion of bronchiectasis associated with abnormalities on chest X ray [n=37] and/or on high resolution computed tomography [HRCT] [n=36]. Mean age at diagnosis was 5 years 9 months; [range: 6 months-14 years]. Persistent cough and bronchorhea were the most common symptoms. Fourteen patients [34%] had dyspnoea on first presentation, 11 of them [26.8%] had chest deformation and/or finger clubbing. Haemoptysis was noted in only two cases. Mean time to diagnosis from symptom onset was 2.7 years [range: 2 months- 4 years]. The underlying aetiologies were identified in 52% of patients. Cystic fibrosis [17%], previous pneumonic illness [9.7%], primary ciliary dyskinesia [9.7%] and immunodeficiency [9.7%] were the most common causes. After a mean follow-up of 6.6 years, the annual lower respiratory infection rate decreased from 7.2 +/- 3 to 3.1 +/- 2.6 [p<0.05], Twenty one point nine per cent of patients had chronic respiratory failure and five patients required surgery. Delays diagnosis of bronchiectasis remains important in our country. Congenital and indeterminate aetiologies are the most common forms. Prognosis is poor with a high prevalence of chronic respiratory failure

Bronquiectasias no asociadas a fibrosis quística / Non-cystic fibrosis bronchiectasis

Las bronquiectasias no asociadas a fibrosis quística (FQ) son causa frecuente de morbimortalidad de acuerdo a la severidad de la patología subyacente. Su diagnóstico precoz en base a la clínica y el scanner, toda vez que se haya descartado una FQ, es clave para el manejo oportuno y sistemático de los distintos tipos asociados a las diversas etiologías que se menciona. Se han realizado avances en el estudio genético de los factores intrínsecos presentes en poblaciones específicas. El manejo adecuado de la patología de base, cuando se descubre, no siempre resulta exitoso y el tratamiento definitivo es motivo de investigación. Por ahora, el uso de antibióticos sistémicos e inhalados más fisioterapia son las únicas herramientas que han demostrado eficacia en mejorar síntomas y calidad de vida en la mayoría de los casos. Se presenta una revisión actualizada de las causas de bronquiectasias no relacionadas con FQ, incidencia y prevalencia, aspectos clínicos más relevantes, junto a las evidencias y controversias sobre su tratamiento.

Childhood bronchiectasis: a review

More than two decades ago, bronchiectasis unrelated to cystic fibrosis [CF] was termed "Orphan disease", because it had become an uncommon clinical entity among children in the developed world. It is more common among children in lower socioeconomic classes and in developing countries, due to more frequent recurrent respiratory infections, environmental airway irritants, poor immunization, and malnutrition. The pathophysiology includes airway inflammation, mucous production, and regional airway obstruction, but the reasons some children develop bronchiectasis while other do not is not clear. Public measures aimed at improving living conditions for children and prevention of respiratory infections with anti-viral vaccines will have a good impact on childhood bronchiectasis than medical treatment

Bronquiectasias: aspectos diagnósticos e terapêuticos Estudo de 170 pacientes / Bronchiectasis: diagnostic and therapeutic features A study of 170 patients

INTRODUÇAO: Bronquiectasias são freqüentemente encontradas na prática médica no Brasil, levando a significativa morbidez e comprometimento da qualidade de vida de seus portadores. OBJETIVOS: Analisar aspectos diagnósticos e terapêuticos em uma série de pacientes com bronquiectasias atendidos em um serviço de doenças pulmonares. MÉTODO: Sinais, sintomas, achados radiográficos e microbiológicos, e resultados terapêuticos foram estudados em 170 pacientes portadores de bronquiectasias hospitalizados no período de 1978 a 2001 - 62,4 por cento do sexo feminino, 37,6 por cento do masculino, com idade média de 37 anos, variando entre 12 e 88 anos. RESULTADOS: Antecedente de pneumonia na infância foi detectado em 52,5 por cento dos pacientes, de tratamento tisiológico em 19,8 por cento; 8,8 por cento tinham asma brônquica, e dois tinham síndrome de Kartagener. Os sintomas mais comuns foram tosse (100 por cento), expectoração (96 por cento) e estertores pulmonares (66 por cento). As lesões eram unilaterais em 46,5 por cento dos casos. Pneumococo, H. influenzae ou flora mista estiveram presentes em 85 por cento das amostras de escarro examinadas. Os 170 pacientes receberam inicialmente tratamento clínico à base de antibióticos e fisioterapia respiratória; 88 deles (52 por cento) mais jovens, com lesões menores e boa reserva funcional foram submetidos à cirurgia de ressecção pulmonar (82 unilaterais e seis bilaterais). Ocorreram dois óbitos hospitalares entre os pacientes que receberam tratamento exclusivamente clínico. Os pacientes tratados cirurgicamente tiveram acentuada melhora dos sintomas, raramente necessitando ser reinternados. CONCLUSÕES: Os prolongados sintomas broncopulmonares foram permanentemente aliviados na maioria dos pacientes com bronquiectasias que puderam ir à cirurgia de ressecção pulmonar, diferentemente dos que seguiram com o tratamento clínico.

Bronquiectasia localizada e multissegmentar: perfil cínico-epidemiológioco e resultado do tratamento cirúrgico em 67 casos / Localized and multisegmental bronchiectasis: clinical-epidemiological profile and surgical treatment results in 67 cases

A bronquiectasia, doença rara no Primeiro Mundo, tem alta prevalência nos paises em desenvolvimento. No Brasil, as principais causas säo infecçöes respioratória, virais ou bacterianas na infância e tuberculose. A antibioticoterapia diminui consideravelmente a morbidade, mas nos pacientes com sintomatologia persistente,a ressecçäo cirúrgica oferece maior possibilidade de cura ou melhor qualidade de vida. OBJETIVOS: Avaliar prospectivamente o perfil clínico-epidemiológico de pacientes com bronquiectasia e o resultado do tratamento cirúrgico em dois hospitais da rede pública de saúde de Fortaleza (CE), na regiäo Nordeste do Brasil. CASUíSTICA E MÉTODO: Foram estudados dados demográficos, etiologia da doença e complicaçöes e sucesso terapêutico da cirurgia de 67 portadores de bronquiectasia operados de agosto de 1989 a março de 1999. RESULTADOS: Os pacientes, 39 mulheres e 28 homens, tinham idade média de 32,5 ñ 14,1 anos e as causas mais freqüentes da bronquiectasia foram infecçöes bacterianas ou virais (44,8 por cento) e tuberculose (31,3 por cento). A doença era localizada em 46 pacientes e multissegmentar em 21, sendo bilateral em seis. Näo houve óbito cirúrgico e as complicaçöes pós-operatórias foram mais freqüentes nos pacientes com bronquiectasia multissegmentar (9/21 versus 6/46, p = 0,011). Dos 62 pacientes com sequimento, 49 foram curados, dez melhoraram e três näo obtiveram benefícios. O resultado foi excelente em 39 pacientes com doença localizada e em dez com doença multissegmentar (p < 0,001). CONCLUSÄO: O resultado mostra dois grupos distintos de pacientes: os com bronquiectasia localizada, que apresentam menos complicaçöes cirúrgica e melhores resultados pós-operatórios, e os com bronquiectasia multissegmentar

Asociación entre bronquiectasias, timoma e hipogammaglobulinemia: síndrome de good / Association between bronchiectasis, thymona and hypogammaglobulinemia: good's a syndrome

Se presenta el caso de un paciente con bronquiectasias, hipogamaglobulinemia y timoma, entidad denominada Sindrome de Good. Mostranmos los aspectos clínicosde laboratorio, radiológicos y funcionales de este caso. Se revisa en la literatura la descripción de casos similares

Eficacia y tolerabilidad de la Esparfloxacina en el tratamiento empírico de las infecciones bacterianas agudas del tracto respiratorio bajo / Effectiveness and tolerabilidad of the esparfloxacina in the empiric treatment of the sharp bacterial infections of the breathing tract under

Se realizó un estuio abierto para el tratamiento empírico de infecciones respiratorias bajas con esparfloxacina en 40 pacientes ambulatorios, atendidos en la Consulta Externa de Neumología del Hospital Nacional Cayetano Heredia (Lima, Perú). La edad promedio fue de 55.2 años (23 a 84 años). 24 (60 por ciento) fueron de sexo femenino. 19 pacientes eran de más de 60 años y de éstos 9 tenían 70 o más años: Sólo dos de los pacientes no tenían una enfermedad pulmonar de base: Uno con bronquitis aguda y el otro con neumonía. 15 pacientes (37.5 por ciento) tuvieron bronquiectasia post-tuberculosas, con una edad promedio de 38.9 años, otros 15 (37.5 por ciento) asma bronquial, con una edad promedio de 61.5 años, 7 (17.5 por ciento) enfermedad pulmonar obstructiva crónica (EPOC) secundaria a tabaquismo y 1 (2.5 por ciento) bronquiectasia secundaria a infecciones bacterianas repetidas. Un paciente tuvo una reacción adversa generalizada por lo que tuvo que suspender el tratamiento. Los otros 39 pacientes evolucionaron satisfactoriamente. Al término del tratamiento 17 pacientes (43.6 por ciento) no tuvieron ninguna sintomatología. Entre los 22 pacientes que quedaron con algún tipo se síntomas 13 habían tenido tuberculosis pulmonar, 6 eran asmáticos y 3 con EPOC secundario a tabaquismo. El puntaje (graduado de 0 a 3) promedio de síntomas por paciente disminuyó de 8.3 con esparfloxacina a 1.15 al término. (p <0.01). La tos disminuyó de un promedio por paciente antes del tratamiento de 1.875 a 0.46 al terminarlo (p < 0.05) la expectoración purulenta de 2.275 a 0.33 por paciente (p <0.05), desapareciendo la fiebre y la hemoptisis en todos los que la tenían. En conclusión, con esparfloxacina se obtiene una respuesta clínico-radiológica altamente eficaz cuando se usa tratamiento empírico de infecciones bacterianas agudas del tracto respiratorio bajo, sobre todo en pacientes con enfermedad pulmonar crónica de base, con buena tolerabilidad y escasos efectos colaterales.