Diagnostic value of nasal nitric oxide for children with primary ciliary dyskinesia / 中华儿科杂志

Objective: To evaluate the value of nasal nitric oxide (nNO) measurement as a diagnostic tool for Chinese patients with primary ciliary dyskinesia (PCD). Methods: This study is a retrospective study. The patients were recruited from those who were admitted to the respiratory Department of Respiratory Medicine, Children's Hospital of Fudan University from March 2018 to September 2022. Children with PCD were included as the PCD group, and children with situs inversus or ambiguus, cystic fibrosis (CF), bronchiectasis, chronic suppurative lung disease and asthma were included as the PCD symptom-similar group. Children who visited the Department of Child health Care and urology in the same hospital from December 2022 to January 2023 were selected as nNO normal control group. nNO was measured during plateau exhalation against resistance in three groups. Mann-Whitney U test was used to analyze the nNO data. The receiver operating characteristic of nNO value for the diagnosis of PCD was plotted and, the area under the curve and Youden index was calculated to find the best cut-off value. Results: nNO was measured in 40 patients with PCD group, 75 PCD symptom-similar group (including 23 cases of situs inversus or ambiguus, 8 cases of CF, 26 cases of bronchiectasis or chronic suppurative lung disease, 18 cases of asthma), and 55 nNO normal controls group. The age of the three groups was respectively 9.7 (6.7,13.4), 9.3 (7.0,13.0) and 9.9 (7.3,13.0) years old. nNO values were significantly lower in children with PCD than in PCD symptom-similar group and nNO normal controls (12 (9,19) vs. 182 (121,222), 209 (165,261) nl/min, U=143.00, 2.00, both P<0.001). In the PCD symptom-similar group, situs inversus or ambiguus, CF, bronchiectasis or chronic suppurative lung disease and asthma were significantly higher than children with PCD (185 (123,218), 97 (52, 132), 154 (31, 202), 266 (202,414) vs. 12 (9,19) nl/min,U=1.00, 9.00, 133.00, 0, all P<0.001). A cut-off value of 84 nl/min could provide the best sensitivity (0.98) and specificity (0.92) with an area under the curve of 0.97 (95%CI 0.95-1.00, P<0.001). Conclusions: nNO value can draw a distinction between patients with PCD and others. A cut-off value of 84 nl/min is recommended for children with PCD.

Anti-Myeloperoxidase (MPO) associated Vasculitis in a young Filipino Male with Bronchiectasis: A case report

Background@#ANCA-associated vasculitis and its subtypes have been associated with pulmonary manifestations, with bronchiectasis being a unique clinical presentation.@*Case Summary@# We report the case of a 26-year-old Filipino male who presented with progressive dyspnea, neuropathic pain, and purpuric rash. Diagnostic evaluation revealed upper lobe bronchiectasis and lower lobe pneumonia, as well as hematuria and proteinuria. ANCA-associated vasculitis (AAV) and tuberculosis were considered. There was improvement of dyspnea, cough and rashes with antibiotics, glucocorticoids (GC), and anti-TB coverage. However, neuropathic pain progressed to the upper and lower extremities with development of weakness. Anti-myeloperoxidase (MPO) Anti-Neutrophil Cytoplasmic Antibody (ANCA) was positive, Electromyography-Nerve Conduction Velocity (EMG-NCV) revealed diffuse sensorimotor axonal polyradiculopathy of both upper and lower extremities. Cyclophosphamide was then given. The patient gradually regained his motor strength while sensory deficits persisted. He was referred to rehabilitation medicine for physical therapy and eventually discharged. This case highlights vasculitis as an associated extrapulmonary manifestation of bronchiectasis, and the possible role of bronchiectasis in the immune-mediated pathogenesis of ANCA- associated vasculitides.

Inmunodeficiencia Común Variable: manifestaciones clínicas iniciales. Serie de casos / Common Variable Immunodeficiency: initial clinical manifestations. Number of cases

La inmunodeficiencia común variable (IDCV) es una inmunodeficiencia primaria caracterizada por hipogammaglobulinemia de comienzo tardío, que se manifiesta principalmente con infecciones recurrentes Objetivo: describir las manifestaciones clínicas iniciales de pacientes con IDCV diagnosticados en el Hospital de Niños Sor Maria Ludovica entre 1981 y 2019.Diecinueve pacientes fueron incluidos. Todos los pacientes tenían historia de infecciones recurrentes, siendo las más frecuentemente observadas la neumonía (74%) y la otitis media (42%).Se documentó diarrea crónica en 9 pacientes (47%), con malabsorción asociada en 6 de ellos. El 32% de los pacientes presentó desnutrición severa y uno de ellos metaplasia gástrica. Un paciente presentó esplenomegalia y otro síndrome de Evans.Bronquiectasias fueron observadas en el 42% de los pacientes al diagnóstico.Conocer las manifestaciones clínicas iniciales de la IDCV es fundamental para el diagnóstico precoz y tratamiento oportuno

Common variable immunodeficiency (CVID) is a primary immunodeficiency characterized by late onset hypogammaglobulinemia, that can manifest as recurrent infections, autoimmunity, digestive disorders and granulomatous disease. Objectives: to describe the initial clinical findings of patients with CVID diagnosed at Hospital de NiñosSorMariaLudovica, between 1981 and 2019. 19 patients were included, 14 were male (74%). All Patients Had a history of recurrent infections, most frequently pneumonia (74%) and acute otitis media (42%). 9 patients suffered from chronic diarrhea (47%), with associated malabsorption in 6 of them. Thirty-two presented with severe malnutrition and 1 patient with gastric metaplasia. One Patient Had Splenomegaly and 1 had Evans´ syndrome. Bronchiectasis were found in 42% of patients at the time of diagnosis. Early suspicion of CVID from pediatricians is essential in order to arrive at a proper diagnosis

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

Heterogeneity of non-cystic-fibrosis bronchiectasis in multiethnic Singapore: A prospective cohort study at a tertiary pulmonology centre

INTRODUCTION@#Non-cystic fibrosis bronchiectasis (NCFB) is a highly heterogenous disease. We describe the clinical characteristics of NCFB patients and evaluate the performance of Bronchiectasis Severity Index (BSI) in predicting mortality.@*METHODS@#Patients attending the bronchiectasis clinic between August 2015 and April 2020 with radiologically proven bronchiectasis on computed tomography were recruited. Clinical characteristics, spirometry, radiology, microbiology and clinical course over a median period of 2.4 years is presented.@*RESULTS@#A total of 168 patients were enrolled in this prospective cohort study. They were predominantly women (67.8%), Chinese (87.5%) and never-smokers (76.9%). Median age of diagnosis was 64 years (interquartile range 56-71) and the most common aetiology was "idiopathic" bronchiectasis (44.6%). Thirty-nine percent had normal spirometries. Compared to female patients, there were more smokers among the male patients (53.8% versus 8.5%, @*CONCLUSION@#The NCFB cohort in Singapore has unique characteristics with sex differences. Over half the patients had a history of haemoptysis. The BSI score is a useful predictor of mortality in our population.

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.

Exercise-induced desaturation in subjects with non-cystic fibrosis bronchiectasis: laboratory-based tests versus field-based exercise tests / Dessaturação induzida pelo exercício em pacientes com bronquiectasia não fibrocística: testes laboratoriais versus testes clínicos de campo

ABSTRACT Objective: To investigate the validity of field walking tests to identify exercise-induced hypoxemia and to compare cardiorespiratory responses and perceived effort between laboratory-based and field-based exercise tests in subjects with bronchiectasis. Methods: This was a cross-sectional study involving 72 non-oxygen-dependent participants (28 men; mean age = 48.3 ± 14.5 years; and mean FEV1 = 54.1 ± 23.4% of the predicted value). The participants underwent cardiopulmonary exercise testing (CPET) on a treadmill and constant work-rate exercise testing (CWRET) on the same day (1 h apart). In another visit, they underwent incremental shuttle walk testing (ISWT) and endurance shuttle walk testing (ESWT; 1 h apart). Desaturation was defined as a reduction in SpO2 ≥ 4% from rest to peak exercise. Results: CPET results were compared with ISWT results, as were CWRET results with ESWT results. There was no difference in the magnitude of desaturation between CPET and ISWT (−7.7 ± 6.3% vs. −6.6 ± 5.6%; p = 0.10) and between CWRET and ESWT (−6.8 ± 5.8% vs. −7.2 ± 6.3%; p = 0.50). The incremental tests showed an agreement in the magnitude of desaturation in the desaturation and no desaturation groups (42 and 14 participants, respectively; p < 0.01), as did the endurance tests (39 and 16 participants; p < 0.01). The magnitude of desaturation was similar among the participants who did or did not reach at least 85% of the maximum predicted HR. Conclusions: Field exercise tests showed good precision to detect desaturation. Field tests might be an alternative to laboratory tests when the clinical question is to investigate exercise-induced desaturation in subjects with bronchiectasis.

RESUMO Objetivo: Investigar a validade dos testes de caminhada de campo para identificar dessaturação durante o exercício, comparando os testes de exercício laboratoriais e clínicos de campo quanto às respostas cardiorrespiratórias e percepção de esforço em indivíduos com bronquiectasia não fibrocística. Métodos: Estudo transversal com 72 participantes não dependentes de oxigênio (28 homens; média de idade: 48,3 ± 14,5 anos; média do VEF1: 54,1 ± 23,4% do previsto). Os participantes foram submetidos ao teste de exercício cardiopulmonar (TECP) incremental em esteira e ao constant work-rate exercise testing (CWRET, teste de exercício com carga constante) em esteira, com intervalo de 1 h. Em outra visita, foram submetidos ao incremental shuttle walk test (ISWT, teste de caminhada incremental) e ao endurance shuttle walk test (ESWT, teste de caminhada de resistência), com intervalo de 1 h. A definição de dessaturação foi uma redução da SpO2 ≥ 4% do repouso ao pico do exercício. Resultados: O TECP e o ISWT resultaram em dessaturação de magnitude comparável (−7,7 ± 6,3% vs. −6,6 ± 5,6%; p = 0,10), assim como o fizeram o CWRET e o ESWT (−6,8 ± 5,8% vs. −7,2 ± 6,3%; p = 0,50). Houve concordância entre o TECP e o ISWT quanto ao número de participantes que apresentaram e não apresentaram dessaturação, respectivamente (42 e 14; p < 0,01), bem como entre o CWRET e o ESWT (39 e 16; p < 0,01). A magnitude da dessaturação foi semelhante nos participantes que atingiram ≥ 85% da FC máxima prevista ou não. Conclusões: Os testes de exercício de campo apresentaram boa precisão para detectar dessaturação. Os testes de campo podem ser uma alternativa aos testes de laboratório quando o objetivo é investigar a dessaturação durante o exercício em indivíduos com bronquiectasia.

Systemic inflammation and oxidative stress in adults with bronchiectasis: association with clinical and functional features

OBJECTIVES: To compare the inflammatory and oxidative stress (OS) states of adults with bronchiectasis with those of healthy controls and correlate inflammatory and OS levels with lung function and physical capacity. METHODS: This study used a cross-sectional design. Seventy-four adults with bronchiectasis (age: 49±15 years, forced expiratory volume in 1 second [FEV1]: 52.5±25.6%) and 42 healthy controls (age: 44±17 years, FEV1: 95.9±14.0%) performed cardiopulmonary exercise tests and incremental shuttle walking tests. Their physical activity in daily life, inflammatory cytokine, and antioxidant levels in plasma were measured. RESULTS: Compared to that of the controls, the levels of interleukin (IL)-6 (p<0.001), IL-10 (p<0.001), carbonylated proteins (p=0.001), and superoxide anions (p=0.046) were significantly increased in adults with bronchiectasis. Catalase activity was also reduced in this group (p<0.001). The inflammatory markers IL-1β, IL-6, and tumor necrosis factor-α correlated negatively with aerobic capacity (r=-0.408, r=-0.308, and r=-0.207, respectively). We observed similar correlations with OS markers (thiobarbituric acid and carbonyls; r=-0.290 and r=0.379, respectively), and these markers also significantly correlated with the aerobic capacity. CONCLUSIONS: Adults with bronchiectasis presented an increased systemic inflammatory response that correlated negatively with physical capacity.

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.

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.

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

Mucosal bacterial vaccines in clinical practice - a novel approach to an old problem?

SUMMARY OBJECTIVES To evaluate the efficacy of mucosal bacterial vaccines (MBV) in reducing the number of exacerbations in patients with chronic respiratory disease. METHODS A prospective cohort study of patients followed at the Pneumology Unit of the University and Hospital Centre of Coimbra, with frequent infectious exacerbations (3 or more) despite the best therapeutic strategies employed. MBV was used as additional therapy. The number of exacerbations 1 year before therapy and 1 year after it were analyzed. RESULTS A sample of 11 individuals, 45.5% male, mean age 62.5 years. Eight patients had non-cystic fibrosis bronchiectasis, 2 COPD (1 on long-term oxygen therapy), and 1 patient with Mounier Kuhn's syndrome. Three patients were on azithromycin, 1 on inhaled colistin, and 2 on inhaled tobramycin. Out of the 11 patients, one presented complication (fever), which led to a suspension of therapy (excluded from results). Of the 10 patients who completed treatment, 5 had bacterial colonization and were submitted to a custom vaccine. The remaining 6 completed the standard composition. The average of infectious exacerbations in the previous year was 4.3 (0.7 with hospitalization). In the year after therapy, the mean number was 1.5 (0.5 with hospitalization). CONCLUSION The results obtained in this study favor the use of bacterial immunostimulation to reduce the frequency of RRIs in patients with chronic respiratory disease.

RESUMO OBJETIVO Avaliar a eficácia de vacinas bacterianas de mucosa (MBV) na redução do número de exacerbações de pacientes com doença respiratória crônica. MÉTODOS Um estudo de coorte prospectivo incluindo pacientes da Unidade de Pneumologia da Universidade e Centro Hospitalar de Coimbra, com exacerbações infecciosas frequentes (3 ou mais), apesar do uso das melhores estratégias terapêuticas. MBVs foram usadas como terapia adicional. O número de exacerbações 1 ano antes da terapia e 1 ano após ela foram analisados. RESULTADOS Amostra incluiu 11 indivíduos, 45,5% do sexo masculino, com média de idade de 62,5 anos. Oito pacientes apresentaram bronquiectasia não relacionada à fibrose cística, 2 DPOC (1 em oxigenoterapia prolongada) e 1 paciente com síndrome de Mounier-Kuhn. Três pacientes estavam sendo medicados com azitromicina, 1 com colistina inalada e 2 com tobramicina inalada. Dos 11 pacientes, um apresentou complicação (febre), o que levou à suspensão da terapia (excluído dos resultados). Dos 10 pacientes que completaram o tratamento, 5 apresentaram colonização bacteriana e receberam uma vacina personalizada. Os 6 restantes foram tratados com a composição padrão. A média de exacerbações infecciosas no ano anterior foi de 4,3 (0,7 com hospitalização). No ano após a terapia, o número médio foi de 1,5 (0,5 com hospitalização). CONCLUSÃO Os resultados obtidos neste estudo favorecem o uso de imunoestimulação bacteriana para reduzir a frequência de infecções respiratórias recorrentes em pacientes com doença respiratória crônica.

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.

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.

Tomografía computada de alta resolución en el diagnóstico de la fibrosis pulmonar idiopática / High resolution computed tomography in the diagnosis of idiopathic pulmonary fibrosis

El patrón de neumonía intersticial usual (NIU) en la tomografía computada de alta resolución (TCAR) tiene un alto grado de correlación con el patrón histológico lo que permite obviar en dichos casos la necesidad de realizar biopsia pulmonar. La exactitud del diagnóstico de NIU en TCAR se basa en la detección de signos específicos de fibrosis como el panal y las densidades reticulares con bronquiolectasias por tracción. La actualización de las guías 2018 de práctica clínica de la ATS/ERS/JRS/ALAT de Fibrosis Pulmonar Idiopática (FPI) y la declaración de consenso de la Sociedad Fleischner proponen una nueva forma de clasificar los patrones tomográficos tomando en cuenta los aspectos antes considerados. Si bien la presencia de panal sigue siendo el hallazgo principal para caracterizar el patrón NIU, la distribución del reticulado intersticial y la presencia de bronquiolectasias por tracción en el contexto clínico especifico puede ser suficiente para realizar el diagnóstico de fibrosis pulmonar idiopática.

Usual interstitial pneumonia pattern (UIP) in high-resolution computed tomography (HRCT) has a high degree of correlation with the histological pattern which makes it possible to obviate in these cases the need for lung biopsy. The accuracy of a UIP diagnosis in HRCT is based on the detection of specific signs of fibrosis such as honeycomb and reticular densities with traction bronchiolectasis. The update of the 2018 Clinical Practice Guidelines of the ATS / ERS / JRS / ALAT of Idiopathic Pulmonary Fibrosis (IPF) and the Fleischner Society White Paper proposed a new way of classifying the tomographic patterns taking into account the aspects previously considered. Although the presence of honeycomb remains the main finding to characterize the UIP pattern, the distribution of interstitial reticulate and the presence of traction bronchiolectasis, in the specific clinical context, may be sufficient to make the diagnosis of idiopathic pulmonary fibrosis.

Guia para o manejo da asma grave 2019 ­ Associação Brasileira de Alergia e Imunologia / Guide to the management of severe asthma 2019 ­ Brazilian Association of Allergy and Immunology

Antes de rotular um paciente como tendo asma grave, é crucial confirmar o diagnóstico da doença e de sua gravidade, além de excluir diagnósticos diferenciais de condições que podem se assemelhar ou se confundir com a asma, tais como: tuberculose, doença pulmonar obstrutiva crônica, disfunção de corda vocal, apneia do sono, bronquiectasia, entre outras. Neste guia são abordados, além dos diagnósticos diferenciais, dados de história clínica e exames laboratoriais que permitem classificar o paciente com relação à evolução da doença (controlado ou não controlado) e, assim, possibilitar a instituição do esquema terapêutico mais apropriado. São apresentadas alternativas terapêuticas disponíveis para a abordagem clínica desses pacientes, incluindo os imunobiológicos.

Prior to labeling a patient as having severe asthma, a crucial step is to confirm the diagnosis and severity as well as to exclude conditions that may resemble or be mistaken for asthma such as tuberculosis, chronic obstructive pulmonary disease, vocal cord dysfunction, sleep apnea, bronchiectasis, among others. In addition to differential diagnoses, this guide addresses clinical history data and laboratory tests that classify the patient regarding disease progression (controlled or uncontrolled asthma), allowing the establishment of an adequate therapeutic plan. Therapeutic options available for the treatment of these patients are detailed, including biologics.

Cuerpo extraño en vía aérea inferior de dos años de evolución: reporte de caso / Foreign body in lower airway of two years of evolution: case repor

La aspiración de un cuerpo extraño es un acontecimiento fatal que puede conducir a la muerte, definido como el ingreso de manera accidental de un objeto, orgánico o inorgánico, a la vía aérea, alojándose ya sea en la laringe, tráquea o bronquios. Este evento es común en la población pediátrica especialmente entre niños de 18 meses a 3 años. El sitio de alojamiento más común es en el bronquio derecho. El diagnóstico tardío de cuerpo extraño en bronquio está relacionado a neumonías recurrentes, bronquiectasias y atelectasias. Presentamos el caso de un paciente de 12 años de edad con antecedentes de sospecha de aspiración de cuerpo extraño en vía aérea desde hace 2 años. Presentando en ese intervalo de tiempo tos productiva de color verde amarillento y persistente, halitosis, neumonías a repetición, y con hipocratismo digital simétrico. Con ayuda de Imágenes por tomografía axial computarizada y broncoscopia virtual se evidencia el cuerpo extraño alojado en bronquio principal izquierdo, revelando el pulmón izquierdo colapsado, hepatizado, con bronquiectasias inferiores, desplazamiento de corazón hacia la izquierda y pulmón derecho sobre distendido. * Médico residente de Tercer año pediatría, UNHA-VS...(AU)

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.