Good / 中南大学学报(医学版)

A patient with thymoma associated immunodeficiency syndrome (Good's syndrome) and bronchiectasis was retrospectively analyzed. Good's syndrome is a rare condition of immunodeficiency that is characterized by thymoma and hypogammaglobulinemia. It is important to bear in mind that Good's syndrome should be included in the differential diagnosis When patients repeatedly visited for bronchiectasis or infection, we should alert to their immune state and history of thymoma. Early screening of immunological status and aggressive correction of immune deficiency are beneficial to improving the prognosis to patients with Good's syndrome.

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

Characterization of the severity of dyspnea in patients with bronchiectasis: correlation with clinical, functional, and tomographic aspects / Caracterização da gravidade de dispneia em pacientes com bronquiectasias: correlação com aspectos clínicos, funcionais e tomográficos

ABSTRACT Objective: To characterize a population of patients with bronchiectasis, correlating clinical, radiological, and functional aspects with the severity of dyspnea. Methods: This was a cross-sectional study involving adult patients with HRCT-confirmed bronchiectasis, categorized according to the severity of dyspnea (as being mildly or severely symptomatic, on the basis of the modified Medical Research Council scale). We correlated the severity of dyspnea with clinical parameters, functional parameters (spirometry values, lung volumes, and DLCO), and CT parameters. Results: We evaluated 114 patients, 47 (41%) of whom were men. The median age (interquartile range) was 42 years (30-55 years). The most common form was idiopathic bronchiectasis. Of the 114 patients, 20 (17.5%) were colonized with Pseudomonas aeruginosa and 59 (51.8%) were under continuous treatment with macrolides. When we applied the Exacerbation in the previous year, FEV1, Age, Colonization, Extension, and Dyspnea score, the severity of dyspnea was categorized as moderate in 54 patients (47.4%), whereas it was categorized as mild in 50 (43.9%) when we applied the Bronchiectasis Severity Index. The most common lung function pattern was one of obstruction, seen in 95 patients (83.3%), and air trapping was seen in 77 patients (68.7%). The prevalence of an obstructive pattern on spirometry was higher among the patients with dyspnea that was more severe, and most functional parameters showed reasonable accuracy in discriminating between levels of dyspnea severity. Conclusions: Patients with bronchiectasis and dyspnea that was more severe had greater functional impairment. The measurement of lung volumes complemented the spirometry data. Because bronchiectasis is a complex, heterogeneous condition, a single variable does not seem to be sufficient to provide an overall characterization of the clinical condition.

RESUMO Objetivo: Caracterizar uma população de portadores de bronquiectasias e correlacionar aspectos clínicos, radiológicos e funcionais com a gravidade da dispneia. Métodos: Estudo transversal realizado em adultos, portadores de bronquiectasias confirmadas por TCAR, categorizados de acordo com a gravidade da dispneia (pacientes pouco e muito sintomáticos), correlacionando-os com seus parâmetros clínicos, funcionais (espirometria, volumes pulmonares e DLCO) e tomográficos. Resultados: Foram avaliados 114 pacientes (47 homens, 41%). A mediana (intervalo interquartil) de idade foi de 42 (30-55) anos. A etiologia mais frequente foi idiopática. Em relação à colonização, 20 pacientes (17,5%) eram colonizados por Pseudomonas aeruginosa e grande parte fazia uso de macrolídeo continuamente (51,8%). Quanto à gravidade da dispneia, ela foi considerada moderada em 54 pacientes (47,4%) pelo escore Exacerbation in previous year, FEV1, Age, Colonization, Extension, and Dyspnea e leve em 50 (43,9%) pelo Bronchiectasis Severity Index. O padrão funcional mais encontrado foi distúrbio ventilatório obstrutivo (em 83%), e 68% apresentavam aprisionamento aéreo. Pacientes com maior grau de dispneia apresentaram maior prevalência de distúrbio obstrutivo na espirometria, e a maioria dos parâmetros funcionais apresentaram acurácia razoável em discriminar a gravidade da dispneia. Conclusões: Pacientes com bronquiectasias e maior gravidade da dispneia apresentaram um comprometimento funcional mais expressivo. A medida de volumes pulmonares complementou os dados da espirometria. Por se tratar de uma condição complexa e heterogênea, uma única variável parece não ser suficiente para caracterizar a condição clínica de forma global.

Diagnostic Yield of Bronchial Washing Fluid Analysis for Hemoptysis in Patients with Bronchiectasis

PURPOSE: Bronchiectasis is the main cause of hemoptysis. When patients with bronchiectasis develop hemoptysis, clinicians often perform bronchoscopy and bronchial washing to obtain samples for microbiological and cytological examinations. Bronchial washing fluids were analyzed from patients with bronchiectasis who developed hemoptysis, and the clinical impacts of these analyses were examined. MATERIALS AND METHODS: A retrospective observational study of patients who underwent fiberoptic bronchoscopy for hemoptysis in Seoul National University Bundang Hospital, a university affiliated tertiary referral hospital, between January 2006 and December 2010 were reviewed. Among them, patients who had bronchiectasis confirmed by computed tomography and had no definite cause of hemoptysis other than bronchiectasis were reviewed. The demographic characteristics, bronchoscopy findings, microbiological data, pathology results and clinical courses of these patients were retrospectively reviewed. RESULTS: A total of 130 patients were reviewed. Bacteria, non-tuberculous mycobacteria (NTM), and Mycobacterium tuberculosis were isolated from bronchial washing fluids of 29.5%, 21.3%, and 0.8% patients, respectively. Suspected causal bacteria were isolated only from bronchial washing fluid in 19 patients, but this analysis led to antibiotics change in only one patient. Of the 27 patients in whom NTM were isolated from bronchial washing fluid, none of these patients took anti-NTM medication during the median follow-up period of 505 days. Malignant cells were not identified in none of the patients. CONCLUSION: Bronchial washing is a useful method to identify microorganisms when patients with bronchiectasis develop hemoptysis. However, these results only minimally affect clinical decisions.

Primary achalasia with pneumoesophagus and bronchopleural fistula causing right lung collapse and bronchiectasis.

A 35-year-old male non-smoker, presented with a history of recurrent respiratory tract infections, associated with intermittent haemoptysis and haematemesis since childhood. His vital parameters were normal with pulse oximetry saturation of 97% on room air. On physical examination, clubbing was observed. Chest auscultation revealed tubular type of bronchial breath sounds over the right hemithorax.

Adesividade e purulência de secreções respiratórias: implicações no transporte mucociliar em pacientes com bronquiectasias / Adhesiveness and purulence of respiratory secretions: implications for mucociliary transport in patients with bronchiectasis

OBJETIVO: Analisar e comparar as propriedades de transporte de secreções respiratórias, classificadas através de parâmetros selecionados, de indivíduos com bronquiectasias não secundárias à fibrose cística. MÉTODOS: Foram avaliadas amostras de muco respiratório, classificadas como com propriedades de superfície adesivas ou não adesivas, assim como com aspecto mucoide ou purulento, de 35 participantes com bronquiectasias não secundárias à fibrose cística, quanto a velocidade relativa de transporte (VRT), deslocamento em máquina simuladora de tosse (MST) e ângulo de contato (AC). Para as comparações propostas, foram utilizados modelos de ANOVA, com nível de significância estabelecido em 5 por cento. RESULTADOS: Houve uma diminuição significativa no deslocamento em MST, assim como um aumento significativo no AC, das amostras adesivas quando comparadas às não adesivas (6,52 ± 1,88 cm vs. 8,93 ± 2,81 cm e 27,08 ± 6,13º vs. 22,53 ± 5,92º, respectivamente; p < 0,05 para ambos). O mesmo ocorreu na comparação entre as amostras purulentas e mucoides (7,57 ± 0,22 cm vs. 9,04 ± 2,48 cm e 25,61 ± 6,12º vs. 21,71 ± 5,89º; p < 0,05 para ambos). Não houve diferença na VRT entre os grupos, embora os valores estivessem diminuídos, independentemente da adesividade (adesivas: 0,81 ± 0,20; não adesivas: 0,68 ± 0,24) ou do aspecto (purulentas: 0,74 ± 0,22; mucoides: 0,82 ± 0,22) das amostras. CONCLUSÕES: A secreção respiratória de pacientes com bronquiectasia apresentou uma diminuição do transporte ciliar. Maior adesividade e purulência favorecem a piora das propriedades de transporte, demonstradas pela diminuição do deslocamento em MST e pelo aumento do AC.

OBJECTIVE: To analyze and compare the transport properties of respiratory secretions, classified by selected parameters, in individuals with bronchiectasis unrelated to cystic fibrosis. METHODS: We collected mucus samples from 35 individuals with bronchiectasis unrelated to cystic fibrosis. The samples were first classified by their surface properties (adhesive or nonadhesive), as well as by their aspect (mucoid or purulent). We then tested the samples regarding relative transport velocity (RTV), displacement in a simulated cough machine (SCM), and contact angle (CA). For the proposed comparisons, we used ANOVA models, with a level of significance set at 5 percent. RESULTS: In comparison with nonadhesive samples, adhesive samples showed significantly less displacement in the SCM, as well as a significantly higher CA (6.52 ± 1.88 cm vs. 8.93 ± 2.81 cm and 27.08 ± 6.13º vs. 22.53 ± 5.92º, respectively; p < 0.05 for both). The same was true in the comparison between purulent and mucoid samples (7.57 ± 0.22 cm vs. 9.04 ± 2.48 cm and 25.61 ± 6.12º vs. 21.71 ± 5.89º; p < 0.05 for both). There were no significant differences in RTV among the groups of samples, although the values were low regardless of the surface properties (adhesive: 0.81 ± 0.20; nonadhesive: 0.68 ± 0.24) or the aspect (purulent: 0.74 ± 0.22; mucoid: CONCLUSIONS: The respiratory secretions of patients with bronchiectasis showed decreased mucociliary transport. Increased adhesiveness and purulence cause the worsening of transport properties, as demonstrated by the lesser displacement in the SCM and the higher CA.

Hemoptise em hospital de referência em pneumologia / Hemoptysis in a referral hospital for pulmonology

OBJETIVO: Determinar as principais causas de hemoptise e classificar esse sintoma quanto ao volume de sangue expectorado em pacientes internados em um hospital de referência em pneumologia. MÉTODOS: Foram incluídos 50 pacientes com hemoptise internados na enfermaria de pneumologia de um hospital geral na cidade do Recife (PE) no período entre julho de 2005 e fevereiro de 2006. Os dados de interesse foram analisados e comparados aos da literatura mundial. RESULTADOS: As infecções foram principais causas de hemoptise - em 39 pacientes (78 por cento) - a maioria delas relacionadas a sequelas de tuberculose pulmonar ou secundárias a tuberculose ativa. Em relação ao grau de hemoptise, as hemoptises moderadas, diagnosticadas em 28 pacientes (56 por cento), foram as mais encontradas. CONCLUSÕES: Nossos resultados sugerem que todos os pacientes com hemoptise devam ser investigados quanto a infecções.

OBJECTIVE: To determine the main causes of hemoptysis and to classify this symptom, in terms of the amount of blood expectorated, in patients hospitalized at a referral hospital for pulmonology. METHODS: The study included 50 patients with hemoptysis admitted to the pulmonology ward of a general hospital in the city of Recife, Brazil, between July of 2005 and February of 2006. The data of interest were analyzed and compared with those in the literature. RESULTS: The most common cause of hemoptysis was infection-in 39 patients (78 percent)-mostly related to tuberculosis sequelae or active tuberculosis. Regarding the severity of hemoptysis, moderate hemoptysis, diagnosed in 28 patients (56 percent), was the most common. CONCLUSIONS: Our results suggest that all patients who present with hemoptysis should be investigated for infection.

Fibrosis quística: diagnóstico tardío en el adulto presentación de caso / Delayed diagnosis of the cystic fibrosis in adulthood: a case report

Se presenta el caso de un paciente blanco de 47 años con cuadros respiratorios a repetición que cedían con tratamiento sintomático o con el uso de antibióticos por vía oral desde la infancia. A los 39 años de edad, comenzó con agudizaciones más frecuentes que requirieron tratamientos con antibióticos e ingresos en varios hospitales de Ciudad de La Habana, y se le diagnosticó Bronquiectasias diseminadas bilaterales e infectadas con Pseudomonas Aeruginosas, secundarias a una posible Tuberculosis adquirida en la antigua URSS a los 21 años. La falta de lesiones hacia los vértices pulmonares en la radiografía de tórax y el hecho de no haber nunca tenido hijos despertó la sospecha clínica de Fibrosis Quística, la cual fue confirmada por los estudios realizados posteriormente. Nuestro propósito al presentar este caso es alertar que esta enfermedad ha dejado de ser una patología exclusiva de Pediatría y que debe sospecharse ante un cuadro florido de bronquiectasias(AU)

The case of a 47 years old white patient, that presented repeated respiratory symptoms that only improve using symptomatic treatment or oral antibiotics. In 2000 when, he was 39 years old their symptoms increased and he required antibiotic intensive treatment and incomes at several hospital in Havana. After that, he was diagnosed with multiple bronchiectasis spread bilateral and infected Pseudomona Auriginosa, that could brings about, TB acquired at URSS when he was 21 years old. In 2008 because of the absence of lesions toward pulmonary apex in the Rx and the fact he couldn't have children aroused the clinical suspicion of Cystic Fibrosis and it was later confirmed by the accomplished studies. Our purpose to present this case is to alert and prevent that this disease has stopped being Pediatric's exclusive pathology and it should be taking into account when there is a complete picture of a multiple spread bronchiectasis(AU)

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.

Espacios aéreos pulmonares anormales por TCAR / Abnormal lung airspaces HRCT

Objetivos: El propósito de este trabajo es revisar y dar claves par el diagnóstico de las distintas entidades que se presentan como espacios aéreos pulmonares anormales. Material y métodos. Se analizaron en forma retrospectiva los archivos de TCAR de nuestra intitución con diagnóstico de espacios aéreos pulmonares anormales. En todos los casos se evaluó la localización, número (único o múltiples), grosor parietal y alteraciones parenquimatosas y mediastínicas asociadas y se los agrupó de acuerdo con sus asociación con distintas enfermedades. Resultados: En base a las características topográficas, los espacios anormales fueron agrupados en : etiología infecciosa (tuberculosis, neumonía por Pneumocistis jiroveci, aspergilosis, hidatidosis, neumonía cavitada, absceso y neumatocele), enfermedades que producen bronquiectasias (fibrosis quística, síndrome de Kartagener, aspergilosis broncopulmonar alérgica), vasculitis (granulomatosis de wegener y artritis reumatoidea), enfisema, neoplasias no primitivas (metástasis cavitadas) tumores primarios (carcinoma epidermoide y bronquioloalveolar) y otras (linfagioleiomiomatosis, histiocitosis de células de Largerhans, secuestro pulmonar y malformación adenoidea quística). Conclusión: Los espacios aéreos pulmonares anormales son un hallazgo frecuente en los estudió por TCAR. Teniendo en cuenta cierta característica de los mismos, es posible arribar a un diagnóstico probable en la mayoría de los casos.

Abnormal air-filled spaces in the lung with HRCT Objetive: The purpose of this paper is to review and give some clues for the diagnosis of the many entities that present as abnormal air spaces at HRCT. Materials and methods: We retrospectively reviewed the archives of HRCT of our institution with diagnosis of different entities that presented with abnormal air spaces. In all cases we evaluated the location, number (unique o multiple), parietal thickness and concomitant compromise ofparenchyma or mediastinum and we clustered them by their association with different illness. Results: Considering the characteristics in HRCT we grouped the abnormal air spaces in: infectious process (tuberculosis, Pneumocistis jiroveci pneumonia, aspergillosis,hydatidosis, cavitated pneumonia, lung abscess and pneumatocele; entities that present with bronchiectasias (cystic fibrosis, Kartagener syndrome, allergic bronchopulmonary aspergillosis), pulmonary vasculitis (Wegener granulomatosis and rheumatoid arthritis); emphysema; metastasis,primary tumors (epidermoid carcinoma and bronchioalveolar carcinoma) and others (lymphangioleiomyomatosis,Langerhans cell histiocytosis, pulmonary sequestration and cystic adenomatoid malformation) Conclusion: The presence of abnormal air spaces in HRCT is very usual. If we know certain characteristics of them, we could get a provable diagnosis in almost all cases.

Acromesomelic dysplasia with bronchiectasis.

We report a rare case of acromesomelic dysplasia with bilateral bronchiectasis and obstructive sleep apnoea. Diagnosis of acromesomelic dysplasia was based on radiographs of whole skeleton.

Etiology and treatment outcomes of massive hemoptysis.

Massive hemoptysis is a life-threatening condition and can lead to asphyxiation. This is a retrospective review of 101 patients hospitalized with massive hemoptysis at Srinagarind Hospital, Khon Kaen, Thailand, between January 1993 and December 2002. The male to female ratio was 2.1:1. The average age was 47.1 (SD 16.8) years. Half the subjects were farmers and three-fourths had an underlying disease; most notably old pulmonary tuberculosis (41.6%). The mean duration of massive hemoptysis was 3.2 (SD 3.7) days. An initial hematocrit < or = 30% was found in 34.6% of patients, and a prolonged prothrombin time in 4.0%, and thrombocytopenia in 2.0%. Chest radiographs revealed unilateral, bilateral lesions and normal lungs in 57.4, 40.6, and 2.0%, respectively. A chest CT was done in 14.8% of patients. Bronchoscopy localized the bleeding and diagnosed the etiology in 19.8%. The most common causes of massive hemoptysis were bronchiectasis (33.7%), active pulmonary tuberculosis (20.8%) and malignancy (10.9%). Patients were grouped by treatment: 1) conservative (88); 2) emergency bronchial artery embolization (7); and, 3) emergency surgery (6). Of the 88 patients in group 1, the bleeding was stopped in 71 (80.7%) and recurred in 4. Of the 7 patients undergoing emergency bronchial artery embolization, the bleeding was stopped in 6 (86%) and recurred in 1. In the 6 patients who underwent emergency surgery, the bleeding was stopped in all and recurred in 1. Recurrent hemoptysis usually arose within 7 days of the first episode and was well controlled with bronchial arterial embolization. The mortality rate was 17.8%. Of the discharged patients, 36.1% had recurrent hemoptysis. Most of them occurred within one month after discharge. We conclude that, the most common cause of massive hemoptysis is benign rahter than malignant disease. Intensive care with conservative treatment should be applied vigorously. Bronchial artery embolization is an excellent, non-surgical alternative to control bleeding, and should be done before specific surgical intervention.