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1.
Ludovica pediátr ; 24(1): 34-39, Ene-Jun 2021.
Article in Spanish | Redbvs, LILACS, Redbvs, BINACIS | ID: biblio-1293221

ABSTRACT

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


Subject(s)
Humans , Child , Adult , Respiratory Tract Infections , Bronchiectasis , Common Variable Immunodeficiency , gamma-Globulins
2.
Cuad. Hosp. Clín ; 62(1): 38-45, jun. 2021. ilus.
Article in Spanish | LILACS | ID: biblio-1284260

ABSTRACT

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


Subject(s)
Humans , Male , Female , Middle Aged , Bronchiectasis , Pulmonary Disease, Chronic Obstructive , Auscultation , Spirometry , Tuberculosis , Dyspnea , Hemoptysis , Metronidazole
3.
J. bras. pneumol ; 47(3): e20210017, 2021. tab, graf
Article in English | LILACS | ID: biblio-1279286

ABSTRACT

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.


Subject(s)
Humans , Female , Adult , Middle Aged , Aged , Pseudomonas Infections/drug therapy , Bronchiectasis/drug therapy , Pseudomonas aeruginosa , Sputum , Brazil , Risk Factors
4.
J. bras. pneumol ; 47(2): e20200134, 2021. tab, graf
Article in English | LILACS | ID: biblio-1154699

ABSTRACT

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.


Subject(s)
Humans , Male , Adult , Middle Aged , Bronchiectasis , Exercise Test , Oxygen Consumption , Forced Expiratory Volume , Cross-Sectional Studies , Walking , Exercise Tolerance , Laboratories
5.
Article in English | WPRIM | ID: wpr-887531

ABSTRACT

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.


Subject(s)
Aged , Bronchiectasis/epidemiology , Cohort Studies , Female , Fibrosis , Humans , Male , Middle Aged , Prospective Studies , Pulmonary Medicine , Severity of Illness Index , Singapore/epidemiology
6.
Article in English | WPRIM | ID: wpr-880663

ABSTRACT

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.


Subject(s)
Agammaglobulinemia/complications , Bronchiectasis/complications , Humans , Retrospective Studies , Thymoma/complications , Thymus Neoplasms/complications
7.
Arch. argent. pediatr ; 118(6): S164-S182, dic 2020. tab, ilus
Article in Spanish | LILACS, BINACIS | ID: biblio-1146240

ABSTRACT

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


Subject(s)
Humans , Male , Female , Child , Bronchiectasis/diagnosis , Signs and Symptoms , Bronchiectasis/complications , Bronchiectasis/etiology , Bronchiectasis/therapy , Bronchiectasis/epidemiology , Aftercare
10.
J. bras. pneumol ; 46(5): e20190162, 2020. tab, graf
Article in English | LILACS | ID: biblio-1134891

ABSTRACT

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.


Subject(s)
Humans , Male , Adult , Bronchiectasis/complications , Tomography, X-Ray Computed/methods , Dyspnea/etiology , Respiratory Function Tests , Spirometry , Severity of Illness Index , Bronchiectasis/epidemiology , Bronchiectasis/diagnostic imaging , Cross-Sectional Studies , Multidetector Computed Tomography
11.
Clinics ; 75: e1698, 2020. tab
Article in English | LILACS | ID: biblio-1101087

ABSTRACT

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.


Subject(s)
Extracorporeal Membrane Oxygenation/methods , Lung Diseases/therapy , Postoperative Complications , Pulmonary Emphysema/therapy , Pulmonary Emphysema/epidemiology , Pulmonary Fibrosis/therapy , Pulmonary Fibrosis/epidemiology , Time Factors , Brazil/epidemiology , Bronchiectasis/therapy , Bronchiectasis/epidemiology , Prospective Studies , Retrospective Studies , Lung Transplantation/methods , Treatment Outcome , Familial Primary Pulmonary Hypertension/therapy , Familial Primary Pulmonary Hypertension/epidemiology , Intensive Care Units , Length of Stay , Lung Diseases/epidemiology
12.
Rev Assoc Med Bras (1992) ; 66(5): 659-665, 2020. tab, graf
Article in English | SES-SP, LILACS, SES-SP | ID: biblio-1136250

ABSTRACT

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.


Subject(s)
Humans , Male , Female , Bronchiectasis , Anti-Bacterial Agents , Bacterial Vaccines , Prospective Studies , Colistin , Azithromycin , Middle Aged
13.
Rev. chil. enferm. respir ; 35(4): 268-274, dic. 2019. tab, graf
Article in Spanish | LILACS | ID: biblio-1092705

ABSTRACT

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.


Subject(s)
Humans , Tomography, X-Ray Computed/methods , Idiopathic Pulmonary Fibrosis/diagnostic imaging , Bronchiectasis/etiology , Bronchiectasis/diagnostic imaging , Idiopathic Pulmonary Fibrosis/complications
14.
Acta pediátr. hondu ; 10(2): 1063-1068, oct. 2019-mar. 2020. ilus
Article in Spanish | LILACS | ID: biblio-1118525

ABSTRACT

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)


Subject(s)
Humans , Male , Child , Bronchiectasis/diagnosis , Foreign-Body Reaction/complications , Pneumonia , Bronchoscopy
15.
Neumol. pediátr. (En línea) ; 14(2): 86-91, jul. 2019. ilus, tab
Article in Spanish | LILACS | ID: biblio-1015004

ABSTRACT

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.


Subject(s)
Humans , Male , Child , Adult , Aspergillosis, Allergic Bronchopulmonary/diagnosis , Aspergillosis, Allergic Bronchopulmonary/therapy , Bronchiectasis/physiopathology , Bronchiectasis/therapy , Cystic Fibrosis/diagnosis , Aspergillosis, Allergic Bronchopulmonary/diagnostic imaging , Bronchiectasis/diagnosis , Bronchiectasis/etiology , Bronchiectasis/epidemiology , Radiography, Thoracic , Macrolides/therapeutic use , Cystic Fibrosis/therapy , Cystic Fibrosis/epidemiology , Anti-Bacterial Agents/therapeutic use
16.
J. bras. pneumol ; 45(4): e20190122, 2019. tab, graf
Article in English | LILACS | ID: biblio-1012570

ABSTRACT

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.


Subject(s)
Humans , Bronchiectasis/therapy , Bronchiectasis/diagnostic imaging , Consensus , Quality of Life , Respiratory Tract Infections/diagnosis , Respiratory Tract Infections/etiology , Respiratory Tract Infections/physiopathology , Respiratory Tract Infections/therapy , Brazil , Bronchiectasis/etiology , Bronchiectasis/physiopathology , Tomography, X-Ray Computed/methods , Chronic Disease , Disease Management
17.
Clinics ; 74: e1399, 2019. tab, graf
Article in English | LILACS | ID: biblio-1039546

ABSTRACT

OBJECTIVE: High-resolution computed tomography (HRCT) allows the early detection of pathological changes in the lung structure, and reproducible scoring systems can be used to quantify chest computed tomography (CT) findings in patients with cystic fibrosis (CF). The aim of the study was to describe early HRCT findings according to a validated scoring system in infants with CF diagnosed by newborn screening (NBS). METHODS: This cross-sectional study included infants with CF diagnosed by NBS who were born between January 2013 and January 2017 and who underwent HRCT scanning within the first year after diagnosis when they were clinically stable. The CT scans were evaluated using the modified Bhalla score. RESULTS: Thirty-two subjects underwent HRCT scanning. The mean total-modified Bhalla score was 3.6±2.1, and 93.8% of the scans were abnormal. Pseudomonas aeruginosa airway colonization was associated with increased modified Bhalla score values. Bronchial wall thickening was the most common feature (90.6%), followed by bronchial collapse/consolidation (59.4%), mosaic attenuation/perfusion (50%), bronchiectasis (37.5%) and mucus plugging (15.6%). Bronchial wall thickening was diffuse in most of the patients. CONCLUSION: A substantial proportion of infants diagnosed with CF after detection by NBS already showed evidence of lung disease. P. aeruginosa colonization was associated with increased Bhalla scores, highlighting the importance of this CF pathogen in early structural lung disease. The presence of bronchial wall thickening at such a young age may reflect the presence of airway inflammatory processes. The detection and quantification of structural abnormalities with the modified Bhalla score may aid in the identification of lung disease before it is clinically apparent.


Subject(s)
Humans , Male , Female , Infant, Newborn , Infant , Neonatal Screening , Cystic Fibrosis/diagnostic imaging , Bronchiectasis/diagnostic imaging , Tomography, X-Ray Computed/methods , Cross-Sectional Studies
18.
Korean Journal of Radiology ; : 1368-1380, 2019.
Article in English | WPRIM | ID: wpr-760302

ABSTRACT

Lung cysts are commonly seen on computed tomography (CT), and cystic lung diseases show a wide disease spectrum. Thus, correct diagnosis of cystic lung diseases is a challenge for radiologists. As the first diagnostic step, cysts should be distinguished from cavities, bullae, pneumatocele, emphysema, honeycombing, and cystic bronchiectasis. Second, cysts can be categorized as single/localized versus multiple/diffuse. Solitary/localized cysts include incidental cysts and congenital cystic diseases. Multiple/diffuse cysts can be further categorized according to the presence or absence of associated radiologic findings. Multiple/diffuse cysts without associated findings include lymphangioleiomyomatosis and Birt-Hogg-Dubé syndrome. Multiple/diffuse cysts may be associated with ground-glass opacity or small nodules. Multiple/diffuse cysts with nodules include Langerhans cell histiocytosis, cystic metastasis, and amyloidosis. Multiple/diffuse cysts with ground-glass opacity include pneumocystis pneumonia, desquamative interstitial pneumonia, and lymphocytic interstitial pneumonia. This stepwise radiologic diagnostic approach can be helpful in reaching a correct diagnosis for various cystic lung diseases.


Subject(s)
Amyloidosis , Birt-Hogg-Dube Syndrome , Bronchiectasis , Diagnosis , Emphysema , Histiocytosis , Histiocytosis, Langerhans-Cell , Lung Diseases , Lung Diseases, Interstitial , Lung , Lymphangioleiomyomatosis , Neoplasm Metastasis , Pneumonia, Pneumocystis
19.
Article in Korean | WPRIM | ID: wpr-760110

ABSTRACT

Woakes' syndrome is a group of disease which include recurrent nasal polyps resulting in the broadening of the nasal pyramid, the onset of hypoplasia of frontal sinus and bronchiectasis, as well as the production of mucous discharge. Children and young adults are mostly susceptible to Woakes' syndrome due to the plasticity of the bone. Even though the exact etiology is unknown, genetic factor is thought to be influential because it is often diagnosed in siblings. Otolaryngologically, the mainstream method of removing nasal polyp by endoscopic sinus surgery as well as topical or systemic treatment can be helpful. We report two siblings who visited our clinic both complaining of nasal obstruction. The patients presented with recurrent nasal polyps and showed signs of bronchiectasis, which led to the diagnosis of Woakes' syndrome. These rare cases are presented here with a review of related literature.


Subject(s)
Bronchiectasis , Child , Diagnosis , Fibrinogen , Frontal Sinus , Humans , Methods , Nasal Obstruction , Nasal Polyps , Plastics , Siblings , Young Adult
20.
Article in English | WPRIM | ID: wpr-759530

ABSTRACT

BACKGROUND: Woake's syndrome (WS) is a recurrent nasal polyposis, accompanied by broadening of the nose, frontal sinus aplasia, dyscrinia, and bronchiectasis. There has been no previous report on anesthetic management in patients with WS. CASE: We describe a case involving a 13-year-old male patient with WS who was scheduled for septorhinoplasty for necrotic ethmoiditis. Anesthesia was induced and maintained with propofol and remifentanil using a target-controlled infusion device. The anesthetic considerations of this rare syndrome and the advantages of an intravenous infusion method over local and volatile anesthesia for these patients are discussed. We report on caveats, such as pulmonary dysfunction during the anesthetic management, and nasal structural problems encountered in WS patients. CONCLUSIONS: Given that conventional inhaled anesthesia reduces ciliary movement and that local anesthesia with sedative has several disadvantages, perioperative control and precautions against respiratory infections by using antibiotics, and preventing cilio-depressant actions, are important for anesthetic management.


Subject(s)
Adolescent , Anesthesia , Anesthesia, Local , Anti-Bacterial Agents , Bronchiectasis , Frontal Sinus , Humans , Infusions, Intravenous , Male , Methods , Nasal Polyps , Nose , Propofol , Respiratory Tract Infections
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