Prevalence and prognostic meaning of interstitial lung abnormalities in remote CT scans of patients with interstitial lung disease treated with antifibrotic therapy.

OBJECTIVES: To describe the prevalence and characteristics of interstitial lung abnormalities (ILA) in CT scans performed prior to the initiation of antifibrotics in a series of patients with interstitial lung disease (ILD), and to identify characteristics apparent on early CT scans that could help to predict outcomes. METHODS: We conducted a retrospective observational study. The original cohort consisted of 101 patients diagnosed with ILD and treated with antifibrotics in a tertiary hospital. Patients were included if they had a thoracic CT scan performed at least one year before initiation of therapy. They were classified radiologically in three groups: without ILA, with radiological ILA and extensive abnormalities. ILA were classified as subpleural fibrotic, subpleural non-fibrotic and non-subpleural. The initial scan and the latest CT scan performed before treatment were read for assessing progression. The relationship between CT findings of fibrosis and the radiological progression rate and mortality were analyzed. RESULTS: We included 50 patients. Only 1 (2%) had a normal CT scan, 25 (50%) had extensive alterations and 24 (48%) had radiological criteria for ILA, a median of 98.2 months before initiation of antifibrotics, of them 18 (75%) had a subpleural fibrotic pattern. Significant bronchiectasis and obvious honeycombing in the lower zones were associated with shorter survival (p = 0.04). Obvious honeycombing in the lower zones was also significantly (p < 0.05) associated with a faster progression rate. CONCLUSIONS: Fibrotic ILAs are frequent in remote scans of patients with clinically relevant ILD, long before they require antifibrotics. Findings of traction bronchiectasis and honeycombing in the earliest scans, even in asymptomatic patients, are related to mortality and progression later on.

Bronchiectasis not due to cystic fibrosis. / Bronquiectasias no debidas a fibrosis quística.

Bronchiectasis is a clinical-radiological condition composed of irreversible bronchial dilation due to inflammation and infection of the airways, which causes respiratory symptoms, usually productive cough and infectious exacerbations. Bronchiectasis can have multiple causes, both pulmonary and extrapulmonary, and its clinical presentation is very heterogenous. Its prevalence is unknown, although up to 35-50% of severe COPD and 25% of severe asthma present them, so their underdiagnosis is evident. Chronic bacterial bronchial infection is common, and Pseudomonas aeruginosa is the pathogen that has been found to imply a worse prognosis. Treatment of bronchiectasis has three fundamental characteristics: it must be multidisciplinary (involvement of several specialties), pyramidal (from primary care to the most specialized units) and multidimensional (management of all aspects that make up the disease).

Fibrosis quística: epidemiología, clínica, diagnóstico y tratamiento / Cystic fibrosis: Epidemiology, clinical manifestations, diagnosis and treatment

La fibrosis quística es una enfermedad genética y multisistémica. La principal comorbilidad en la edad adulta es la afectación respiratoria, con la presencia de bronquiectasias, infección bronquial crónica y obstrucción al flujo aéreo. Hasta hace una década los tratamientos estaban dirigidos a favorecer el drenaje de secreciones, reducir las exacerbaciones respiratorias, controlar la infección bronquial crónica y enlentecer el deterioro funcional, pero con la llegada de los moduladores del gen cystic fibrosis transmembrane conductance regulator (CFTR) el paradigma de la fibrosis quística se ha modificado. Este novedoso tratamiento da un paso más frente al tratamiento de esta enfermedad, es capaz de mejorar la producción de proteína CFTR defectuosa y aumentar su expresión en la superficie celular, para así conseguir un mejor funcionamiento del intercambio iónico fluidificando las secreciones respiratorias y reduciendo la obstrucción al flujo aéreo. Además, en la actualidad hay diferentes líneas de investigación orientadas a corregir el defecto genético causante de la fibrosis quística (AU)

Cystic fibrosis is a genetic and multisystemic disease. The main comorbidity in adulthood is respiratory involvement, with the presence of bronchiectasis, chronic bronchial infection and airflow obstruction. Until a decade ago, treatments were aimed at favoring secretion drainage, reducing respiratory exacerbations, controlling chronic bronchial infection and slowing functional deterioration, but with the advent of cystic fibrosis transmembrane conductance regulator (CFTR) modulators, the cystic fibrosis paradigm has changed. This novel treatment goes a step further in the management of this disease, it is able to improve the production of defective CFTR protein and increase its expression on the cell surface, thus achieving a better functioning of ion exchange, fluidizing respiratory secretions and reducing airflow obstruction. In addition, there are currently different lines of research aimed at correcting the genetic defect that causes cystic fibrosis (AU)

Impacto de un programa de rehabilitación respiratoria domiciliario en la calidad de vida y ansiedad/depresión de pacientes con bronquiectasias no fibrosis quística / Impact of a home respiratory rehabilitation program on the quality of life and anxiety/depression of patients with bronchiectasia not cystic fibrosis

Introducción y objetivos: La valoración del efecto de la rehabilitación respiratoria domiciliaria (PRRD) en bronquiectasias no fibrosis quística (BQ no FQ) es un campo poco explorado hasta la fecha. El objetivo fue evaluar cómo influye un PRRD piloto en la disnea, la calidad de vida y en los trastornos del estado de ánimo así como su relación con la gravedad de la enfermedad. Material y métodos: Ensayo clínico no farmacológico en pacientes con BQ no FQ del Hospital Universitario Virgen Macarena. Se aleatorizó en: 1) grupo estudio (GE): programa de entrenamiento (resistencia y fuerza) en domicilio durante 8 semanas, 2) grupo control (GC): medidas educacionales por escrito. Se evaluó la gravedad de la enfermedad con el E-FACED, síntomas (cuestionario de Leicester (LCQ) y disnea (escala mMRC)), la calidad de vida (cuestionario de enfermedades respiratorias de Saint George (SGRQ)) y ansiedad y depresión (cuestionario hospitalario de ansiedad y depresión (HADS). Resultados: Después de 8 semanas en el GE existió mejoría en disnea de 0,46 ± 0,80, p = 0,010 y en la esfera física del LCQ de -0,68 ± 1,2, p = 0,043. Se produjo una mejoría en SGRQ actividad (-9 puntos, p = 0,025) y en el SGRQ total un cambio clínicamente relevante (-7 puntos, p = 0,063). La escala de depresión descendió 2,3 ± 4,2 puntos, p = 0,044. La gravedad no se relacionó con ninguna variable. Conclusiones: El PRRD mostró un claro beneficio en calidad de vida, síntomas y depresión de nuestros pacientes con BQ no FQ. (AU)

Introduction and objectives: the assessment of the effect of home-based pulmonary rehabilitation programmes (HPRP) in noncystic fibrosis bronchiectasis (non-CF BQ) is a field that has been little explored to date. Our objective was to evaluate how a pilot HPRP influences dyspnoea, quality of life and mood disorders and their relationship the severity of the disease. Material and methods: we present non-pharmacological clinical trial in patients with non-CF BQ at the Virgen Macarena University Hospital. It was randomized into1) study group (SG): received training program (resistance and strength) at home for 8 weeks and 2) control group (CG): received written educational measures. We assessed the impact of the program on disease severity (E-FACED), symptoms (Leicester Questionnaire (LCQ) and dyspnea (mMRC scale)), and quality of life (Saint George RespiratoryQuestionnaire) and anxiety and depression (Anxiety and Depression Hospital (HAD)). Results: after 8 weeks there was an improvement in dyspnoea of 0.46 ± 0,80, p = 0.010 and in the physical sphere of the LCQ of -0.68 ± 1.2, p = 0.043.There was an improvement in SGRQ activity (-9 points, p = 0.025) and in the total SGRQ a clinically relevant change (-7 points, p = 0.063).The depression scale decreased 2.3 ± 4.2 points, p = 0.044. There was no relationship between severity and any of the variables studied. Conclusions: the PRRD showed a clear benefit in quality of life, symptoms and depression of our patients with non-CF BQ. KeywordsMesH: Non-cystic fibrosis bronchiectasis, homebased respiratory rehabilitation, impact quality of life. (AU)

Efectividad del tratamiento con ciprofloxacino durante 21 días en la primoinfección por pseudomonas aeruginosa en las bronquiectasias no fibrosis quística / Effectiveness of treatment with ciprofloxacin during 21 days in primoinfection by pseudomonas aeruginosa in the bronchiectasia not cystic fibrosis

Las bronquiectasias no debidas a fibrosis quística (FQ) constituyen la tercera patología inflamatoria crónica más frecuente de las vías respiratorias.La infección bronquial determina la progresión de la enfermedad, siendo la infección por Pseudomonas aeruginosa la que se asocia con peor pronóstico. Por este motivo, las guías de práctica clínica recomiendan la erradicación de P. aeruginosa en la infección primaria. Hasta el momento ningún estudio ha demostrado la utilidad real de esta pauta de tratamiento en el manejo de la infección bronquial inicial por Pseudomonas aeruginosa, por lo que el objetivo de este estudio es determinar la efectividad del tratamiento con Ciprofloxacino 750 mg cada 12 horas por vía oral durante 21 días en la erradicación de P. aeruginosa en pacientes con bronquiectasias no relacionadas con FQ. (AU)

Bronchiectasis not due to cystic fibrosis (CF) constitutes the third most frequent chronic inflammatory pathology of the airways. Bronchial infection determines the progression of the disease, being infection by Pseudomonas aeruginosa the one that is associated with the worst prognosis. For this reason, clinical practice guidelinesrecommend eradication of P. aeruginosa in primary infection. At the moment, any study has shown the real usefulness of this treatment regimen in the management of the initial bronchial infection by Pseudomonas aeruginosa, so the objective of this study is to determine the effectiveness of treatment with Ciprofloxacin 750 mg every 12 hours orally for 21 days in the eradication of P. aeruginosa in patients with non-CF bronchiectasis. (AU)

Mycobacterial infection after COVID-19 pneumonia / Infección por micobacterias tras neumonía por COVID-19

A 69-year-old man with a history of hypertrophic cardiomyopathy and major depressive disorder was admitted to the Emergency Department with fever, weakness, and shortness of breath. He was diagnosed with acute respiratory distress syndrome due to COVID-19 and received oxygen and steroids during a one-month hospital stay. After discharge, he continued steroids and home oxygen therapy for nearly two years. CT scans revealed bronchiectasis and ground glass opacities related to COVID-19. He developed pulmonary nodules and M. intracellulare infection, which were treated with rifampicin, ethambutol, and azithromycin. After six months of treatment, the patient showed clinical and radiological improvement (AU)

Un hombre de 69 años con antecedentes de miocardiopatía hipertrófica y trastorno depresivo mayor acudió a urgencias por fiebre, debilidad y dificultad respiratoria. Se le diagnosticó síndrome de distrés respiratorio agudo debido a COVID-19 y fue ingresado en planta de neumología, donde recibió oxígeno y esteroides durante 1 mes. Después del alta continuó con esteroides y oxigenoterapia domiciliaria durante casi 2 años. Las tomografías objetivaron bronquiectasias y opacidades en vidrio deslustrado relacionadas con la COVID-19. Desarrolló nódulos pulmonares e infección por Mycobacterium intracellulare, siendo tratado con rifampicina, etambutol y azitromicina. Después de 6 meses de tratamiento, el paciente mostró mejoría clínica y radiológica (AU)

Cystic fibrosis: Epidemiology, clinical manifestations, diagnosis and treatment. / Fibrosis quística: epidemiología, clínica, diagnóstico y tratamiento.

Cystic fibrosis is a genetic and multisystemic disease. The main comorbidity in adulthood is respiratory involvement, with the presence of bronchiectasis, chronic bronchial infection and airflow obstruction. Until a decade ago, treatments were aimed at favoring secretion drainage, reducing respiratory exacerbations, controlling chronic bronchial infection and slowing functional deterioration, but with the advent of cystic fibrosis transmembrane conductance regulator (CFTR) modulators, the cystic fibrosis paradigm has changed. This novel treatment goes a step further in the management of this disease, it is able to improve the production of defective CFTR protein and increase its expression on the cell surface, thus achieving a better functioning of ion exchange, fluidizing respiratory secretions and reducing airflow obstruction. In addition, there are currently different lines of research aimed at correcting the genetic defect that causes cystic fibrosis.

Efectividad del suero salino hipertónico nebulizado en pacientes con hipersecreción bronquial / Effectiveness of nebulized hypertonic saline in patients with bronchial hypersecretion

Introducción: El suero salino hipertónico (SSH) nebulizado mejora la calidad de vida y reduce las exacerbaciones en pacientes con fibrosis quística. Se desconoce si ofrecería los mismos beneficios en otras patologías hipersecretoras.MétodosEstudio observacional retrospectivo. Se incluyeron pacientes que superaron la prueba de tolerancia e iniciaron SSH al 5,8% con un año de seguimiento. Se cuantificaron parámetros clínicos y asistenciales en los años previo y posterior al inicio del tratamiento.ResultadosUn total de 101 pacientes, 60,4% mujeres, media de edad 65años (IC95%: 62,4-67,9): 82 (81,2%) bronquiectasias, 6 (5,9%) EPOC, 2 (2%) asma, 1 (1%) EPID y 10 (9,9%) otros. Se evidenció una reducción de la broncorrea (91,1% vs 75,2%), de las infecciones de repetición (57,4% vs 22,8%) y de los ciclos de antibioterapia (1,54 vs 0,55), así como un aumento del FEV1 (1.881ml vs 1.942ml) y una disminución de las asistencias a atención primaria (2,94 vs 1,1), de urgencias (0,36 vs 0,17) y de hospitalizaciones (0,17 vs 0,06). Setenta y tres pacientes (72,3%) presentaron una adecuada tolerancia.ConclusiónLa nebulización de SSH al 5,8% en pacientes con hipersecreción bronquial es segura y tiene un destacable impacto clínico y asistencial. (AU)

Introduction: Nebulized hypertonic saline (HS) improves quality of life and reduces exacerbations in patients with cystic fibrosis. It is unknown if it would offer the same benefits in other hypersecretory pathologies.MethodsRetrospective observational study. Patients who passed the tolerance test and started HS 5.8% with one year of follow-up were included. Clinical and healthcare parameters were quantified in the year before and after the start of treatment.Results101 patients, 60.4% women, 65years (95%CI: 62.4-67.9): 82 (81.2%) bronchiectasis, 6 (5.9%) COPD, 2 (2%) asthma, 1 (1%) ILD, and 10 (9.9%) other causes. There was a reduction in bronchorrhea (91.1% vs 75.2%), recurrent infections (57.4% vs 22.8%) and cycles of antibiotic therapy (1.54 vs 0.55), as well as an increase in FEV1 (1881ml vs. 1942ml) and a decrease in visits to primary care (2.94 vs. 1.1), emergencies (0.36 vs. 0.17) and hospitalizations (0.17 vs. 0.17). 06). 73 patients (72.3%) presented an adequate tolerance.ConclusionNebulization of HS 5.8% in patients with bronchial hypersecretion is safe and has a remarkable clinical and healthcare impact. (AU)

[Effectiveness of nebulized hypertonic saline in patients with bronchial hypersecretion]. / Efectividad del suero salino hipertónico nebulizado en pacientes con hipersecreción bronquial.

INTRODUCTION: Nebulized hypertonic saline (HS) improves quality of life and reduces exacerbations in patients with cystic fibrosis. It is unknown if it would offer the same benefits in other hypersecretory pathologies. METHODS: Retrospective observational study. Patients who passed the tolerance test and started HS 5.8% with one year of follow-up were included. Clinical and healthcare parameters were quantified in the year before and after the start of treatment. RESULTS: 101 patients, 60.4% women, 65years (95%CI: 62.4-67.9): 82 (81.2%) bronchiectasis, 6 (5.9%) COPD, 2 (2%) asthma, 1 (1%) ILD, and 10 (9.9%) other causes. There was a reduction in bronchorrhea (91.1% vs 75.2%), recurrent infections (57.4% vs 22.8%) and cycles of antibiotic therapy (1.54 vs 0.55), as well as an increase in FEV1 (1881ml vs. 1942ml) and a decrease in visits to primary care (2.94 vs. 1.1), emergencies (0.36 vs. 0.17) and hospitalizations (0.17 vs. 0.17). 06). 73 patients (72.3%) presented an adequate tolerance. CONCLUSION: Nebulization of HS 5.8% in patients with bronchial hypersecretion is safe and has a remarkable clinical and healthcare impact.

Biomarcadores biológicos en las enfermedades respiratorias / Biological Biomarkers in Respiratory Diseases

En los últimos años la llamada «medicina personalizada o de precisión» ha irrumpido con fuerza en el manejo de las enfermedades, entre ellas las respiratorias. La posibilidad de implantar esta forma de trabajar pasa indefectiblemente por el hallazgo y validación de biomarcadores biológicos que se relacionen bien con el diagnóstico, tratamiento o pronóstico de los pacientes respiratorios. En este sentido, la mayoría de enfermedades respiratorias o grupo de las mismas ya cuentan con biomarcadores biológicos de mayor o menor fiabilidad, y se están realizando un gran número de estudios en busca de nuevos de estos indicadores. El objetivo de la presente revisión es poner al día al lector y analizar la literatura científica existente sobre la existencia y validez diagnóstica, terapéutica o pronóstica de los biomarcadores biológicos más importantes en la actualidad en las principales enfermedades respiratorias, así como sobre los retos futuros en este sentido. (AU)

In recent years, personalized or precision medicine has made effective inroads into the management of diseases, including respiratory diseases. The route to implementing this approach must invariably start with the identification and validation of biological biomarkers that are closely related to the diagnosis, treatment, and prognosis of respiratory patients. In this respect, biological biomarkers of greater or lesser reliability have been identified for most respiratory diseases and disease classes, and a large number of studies are being conducted in the search for new indicators. The aim of this review is to update the reader and to analyze the existing scientific literature on the existence and diagnostic, therapeutic, and prognostic validity of the most important biological biomarkers in the main respiratory diseases, and to identify future challenges in this area. (AU)

[Translated article] Biological Biomarkers in Respiratory Diseases / Biomarcadores biológicos en las enfermedades respiratorias

In recent years, personalized or precision medicine has made effective inroads into the management of diseases, including respiratory diseases. The route to implementing this approach must invariably start with the identification and validation of biological biomarkers that are closely related to the diagnosis, treatment, and prognosis of respiratory patients. In this respect, biological biomarkers of greater or lesser reliability have been identified for most respiratory diseases and disease classes, and a large number of studies are being conducted in the search for new indicators. The aim of this review is to update the reader and to analyze the existing scientific literature on the existence and diagnostic, therapeutic, and prognostic validity of the most important biological biomarkers in the main respiratory diseases, and to identify future challenges in this area. (AU)

En los últimos años la llamada «medicina personalizada o de precisión» ha irrumpido con fuerza en el manejo de las enfermedades, entre ellas las respiratorias. La posibilidad de implantar esta forma de trabajar pasa indefectiblemente por el hallazgo y validación de biomarcadores biológicos que se relacionen bien con el diagnóstico, tratamiento o pronóstico de los pacientes respiratorios. En este sentido, la mayoría de enfermedades respiratorias o grupo de las mismas ya cuentan con biomarcadores biológicos de mayor o menor fiabilidad, y se están realizando un gran número de estudios en busca de nuevos de estos indicadores. El objetivo de la presente revisión es poner al día al lector y analizar la literatura científica existente sobre la existencia y validez diagnóstica, terapéutica o pronóstica de los biomarcadores biológicos más importantes en la actualidad en las principales enfermedades respiratorias, así como sobre los retos futuros en este sentido. (AU)

Biological Biomarkers in Respiratory Diseases. / Biomarcadores biológicos en las enfermedades respiratorias.

In recent years, personalized or precision medicine has made effective inroads into the management of diseases, including respiratory diseases. The route to implementing this approach must invariably start with the identification and validation of biological biomarkers that are closely related to the diagnosis, treatment, and prognosis of respiratory patients. In this respect, biological biomarkers of greater or lesser reliability have been identified for most respiratory diseases and disease classes, and a large number of studies are being conducted in the search for new indicators. The aim of this review is to update the reader and to analyze the existing scientific literature on the existence and diagnostic, therapeutic, and prognostic validity of the most important biological biomarkers in the main respiratory diseases, and to identify future challenges in this area.

Cystic lung disease.

The term cystic lung disease encompasses a heterogeneous group of entities characterised by round lung lesions that correspond to cysts with fine walls, which usually contain air. The differential diagnosis of these lesions can be challenging, requiring both clinical and radiological perspectives. Entities such as pulmonary emphysema and cystic bronchiectasis can simulate cystic disease. High-resolution computed tomography (HRCT) is the imaging technique of choice for the evaluation and diagnosis of cystic lung disease, because it confirms the presence of lung disease and establishes the correct diagnosis of the associated complications. In many cases, the diagnosis can be established based on the HRCT findings, thus making histologic confirmation unnecessary. For these reasons, radiologists need to be familiar with the different presentations of these entities. A wide variety of diseases are characterised by the presence of diffuse pulmonary cysts. Among these, the most common are lymphangioleiomyomatosis, which may or may not be associated with tuberous sclerosis, Langerhans cell histiocytosis, and lymphocytic interstitial pneumonia. Other, less common entities include Birt-Hogg-Dubé syndrome, amyloidosis, and light-chain deposit disease. This article describes the characteristics and presentations of some of these entities, emphasizing the details that can help differentiate among them.

Errores innatos de la inmunidad en adultos / Inborn errors of immunity in adults

Introducción: La inmunodeficiencia común variable es un error innato de la inmunidad que tiene su pico de incidencia en la edad adulta. Se caracteriza por una susceptibilidad aumentada a padecer infecciones respiratorias, autoinmunidad y malignidad, secundario a un estado de hipogammaglobulinemia e inmunodisregulación, causado por mutaciones e interacciones genéticas parcialmente comprendidas. El diagnóstico es de exclusión, tiene una gran heterogeneidad clínica y comúnmente es diagnosticado de forma errónea. Objetivo: Describir un caso clínico de un paciente afectado por un error innato de la inmunidad. Caso clínico: Hombre de 35 años que se presenta a la consulta de Medicina Interna - Inmunología refiriendo un cuadro clínico de 3 años de evolución consistente en múltiples episodios de infecciones sino-pulmonares en los últimos meses, presentaba tos productiva, dificultad respiratoria y pérdida de peso no intencional de aproximadamente 8 kg. Conclusiones: La inmunodeficiencia común variable debe considerarse dentro de los diagnósticos diferenciales en todo paciente que presente alguna de sus manifestaciones clínicas, principalmente aquellas relacionadas con infecciones respiratorias a repetición, antecedente que el paciente puede presentar como relevante en sus consultas de primer nivel con medicina general o con especialistas. Su aproximación diagnóstica consiste en la solicitud de niveles séricos de inmunoglobulinas, prueba de laboratorio de fácil acceso para cualquier clínico independiente de su nivel de atención y su tratamiento se fundamenta en la administración periódica de inmunoglobulina humana exógena de forma endovenosa o subcutánea(AU)

Introduction: Common variable immunodeficiency is an inborn error of immunity that has its peak incidence in adulthood. It is characterized by an increased susceptibility to respiratory infections, autoimmunity and malignancy, secondary to a state of hypogammaglobulinemia and immunodysregulation, caused by mutations and partially understood genetic interactions. The diagnosis is one of exclusion, has great clinical heterogeneity and is commonly misinterpreted. Objective: To describe a clinical case of a patient affected by an inborn error of immunity. Methods: Retrospective description of a case report. Conclusions: Common variable immunodeficiency disorder should be considered within the differential diagnoses in every patient who presents any of its clinical manifestations, mainly those related to recurrent respiratory infections, an antecedent that the patient may present as relevant during the first-level consultations with general medicine physicians or with specialists. Its diagnostic approach consists in measuring serum immunoglobulin levels, an easily accessible laboratory test for any clinic physician regardless of their healthcare level, while its treatment is based on the periodic administration of exogenous human immunoglobulin intravenously or subcutaneously(AU)

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

Effects of Controlled Voluntary Increase in the Ventilatory Demand on Respiratory System Resistance in Healthy and Non-Cystic Fibrosis Bronchiectasis Subjects: A Cross-Sectional Study / Efectos del aumento voluntario y controlado de la demanda ventilatoria en la resistencia del sistema respiratorio en sujetos sanos y en pacientes con bronquiectasias no debidas a fibrosis quística: un estudio transversal

Introduction: Bronchiectasis patients may present a reduced functional capacity due to an increase in the ventilatory demand during exercise. Objective: To evaluate the effects of controlled voluntary hyperinflation and increased respiratory rate on the mechanics of the respiratory system, simulating what happens during exercise, in bronchiectasis and healthy subjects. Methods: Bronchiectasis (n=30) and healthy (n=16) subjects were evaluated by impulse oscillometry (IOS) during a baseline condition, and in controlled conditions with baseline (b) tidal volume (V) and hyperinflation (H), with respiratory rates at 30(R30) and 40(R40) bpm, in a random order. The mixed effects and a significance level at 0.05 were used for comparisons. Results: Resistance at 5Hz (R5), and at minus 20Hz (R5–R20), in kPa/L/s, were higher in subjects with bronchiectasis in all experimental conditions (p<0.05). For the bronchiectasis group, R5 and R5-20 increased with R increase at V (VRb versus VR30 and VR40; VR30 versus VR40; R5, R20 and R5-20 increased with R increase at H (HRb versus HR40; HR30 versus HR40). For the same R, there was a decrease with H compared to V (HRb versus VR30 and VR40; and HR30 versus VR30 and VR40). For the healthy group, only R20 showed differences (HR30 versus HR40; HR40 versus VR40). Conclusion: The tachypnea increases the resistance and reactance of the respiratory system in bronchiectasis patients, and the voluntary hyperinflation caused attenuates this increase. These results can guide the development of strategies to reduce the limitation of physical activity in patients with bronchiectasis. (AU)

Introducción: Los pacientes con bronquiectasias pueden presentar una capacidad funcional reducida debido a un aumento en la demanda ventilatoria durante el ejercicio. Objetivo: Evaluar los efectos de la hiperinsuflación voluntaria y controlada y el aumento de la frecuencia respiratoria en la mecánica del sistema respiratorio, simulando lo que sucede durante el ejercicio, en sujetos sanos y en pacientes con bronquiectasias. Métodos: Se evaluó a sujetos con bronquiectasia (n=30) y sujetos sanos (n=16) mediante la oscilometría de impulso (IOS) en situación basal, y en condiciones controladas con basal (b), volumen corriente (V) e hiperinsuflación (H), con frecuencias respiratorias a 30 (R30) y 40 (R40) bpm, en orden aleatorio. Para las comparaciones se utilizaron el modelo de efectos mixtos y un nivel de significación de 0,05. Resultados: La resistencia a 5Hz (R5) y la diferencia con 20Hz (R5-R20), medida en kPa/l/s, fue mayor en sujetos con bronquiectasias en todas las condiciones experimentales (p<0,05). Para el grupo de bronquiectasias, R5 y R5-R20 aumentaron con el aumento de R en V (VRb versus VR30 y VR40; VR30 versus VR40; R5, R20 y R5-R20 aumentaron con el aumento de R en H (HRb versus HR40; HR30 versus HR40. Para el mismo R, hubo una disminución de H en comparación con V (HRb versus VR30 y VR40; y HR30 versus VR30 y VR40). Para el grupo sano, solo R20 mostró diferencias (HR30 versus HR40; HR40 versus VR40). Conclusión: La taquipnea aumenta la resistencia y la reactancia del sistema respiratorio en pacientes con bronquiectasias, y la hiperinsuflación voluntaria generada atenúa este aumento. Estos resultados pueden guiar el desarrollo de estrategias para reducir la limitación de la actividad física en pacientes con bronquiectasia. (AU)

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

Effects of Controlled Voluntary Increase in the Ventilatory Demand on Respiratory System Resistance in Healthy and Non-Cystic Fibrosis Bronchiectasis Subjects: A Cross-Sectional Study.

INTRODUCTION: Bronchiectasis patients may present a reduced functional capacity due to an increase in the ventilatory demand during exercise. OBJECTIVE: To evaluate the effects of controlled voluntary hyperinflation and increased respiratory rate on the mechanics of the respiratory system, simulating what happens during exercise, in bronchiectasis and healthy subjects. METHODS: Bronchiectasis (n=30) and healthy (n=16) subjects were evaluated by impulse oscillometry (IOS) during a baseline condition, and in controlled conditions with baseline (b) tidal volume (V) and hyperinflation (H), with respiratory rates at 30(R30) and 40(R40) bpm, in a random order. The mixed effects and a significance level at 0.05 were used for comparisons. RESULTS: Resistance at 5Hz (R5), and at minus 20Hz (R5-R20), in kPa/L/s, were higher in subjects with bronchiectasis in all experimental conditions (p<0.05). For the bronchiectasis group, R5 and R5-20 increased with R increase at V (VRb versus VR30 and VR40; VR30 versus VR40; R5, R20 and R5-20 increased with R increase at H (HRb versus HR40; HR30 versus HR40). For the same R, there was a decrease with H compared to V (HRb versus VR30 and VR40; and HR30 versus VR30 and VR40). For the healthy group, only R20 showed differences (HR30 versus HR40; HR40 versus VR40). CONCLUSION: The tachypnea increases the resistance and reactance of the respiratory system in bronchiectasis patients, and the voluntary hyperinflation caused attenuates this increase. These results can guide the development of strategies to reduce the limitation of physical activity in patients with bronchiectasis.