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BACKGROUND AND OBJECTIVES: Cardiopulmonary and skeletal muscle impairment and poor physical activity are potential contributors to reduced functional capacity in cystic fibrosis (CF). The Glittre-ADL test (TGlittre) has great potential for clinical use in adult CF adults, as it meets the need for a comprehensive assessment of physical function using tasks similar to activities of daily living. This study aimed to evaluate the performance of TGlittre in CF adults compared to the 6-min walk test (6MWT) and, secondarily, to quantify the associations of their results with pulmonary function, muscle strength, and health-related quality of life (HRQoL). METHODS: This cross-sectional study evaluated 34 CF adults and compared them with 34 subjects from a control group. The participants underwent the following assessments: functional capacity using TGlittre and 6MWT; spirometry; respiratory muscle strength; handgrip strength (HGS); and HRQoL using the Cystic Fibrosis Questionnaire-Revised (CFQ-R). RESULTS: While CF patients showed a longer time to perform TGlittre compared to controls (134 (119-150) versus 107 (95-126) % of the predicted time p = 0.0002), no difference between these groups was observed in the 6MWT. When the second TGlittre was compared to the first TGlittre, there was a significant decrease in total time for both CF patients (p < 0.0001) and controls (p = 0.0001). TGlittre time correlated with 6MWT distance (6MWD) (rs = -0.641, p < 0.0001), HGS (rs = -0.364, p = 0.034), peripheral oxygen saturation at the end of the test (rs = -0.463, p = 0.006) and the "digestive symptoms" domain of CFQ-R (rs = 0.376, p = 0.028). TGlittre time was shorter in patients who engaged in regular physical activity (3.10 (2.49-3.39) min versus 3.28 (2.95-3.53) min, p = 0.016). CONCLUSIONS: TGlittre is more effective than the 6MWT in detecting limitations during exercise. There is an important learning effect of TGlittre in adult CF patients. TGlittre time was correlated with 6MWD, HGS, oxygen saturation level, and the patient's level of physical activity.
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Fibrosis Quística , Prueba de Esfuerzo , Adulto , Humanos , Prueba de Esfuerzo/métodos , Actividades Cotidianas , Fuerza de la Mano , Fibrosis Quística/diagnóstico , Estudios Transversales , Calidad de VidaRESUMEN
COPD, one of world's leading contributors to morbidity and mortality, is characterized by airflow limitation and heterogeneous clinical features. Three main phenotypes are proposed: overlapping asthma/COPD (ACO), exacerbator, and emphysema. Disease severity can be classified as mild, moderate, severe, and very severe. The molecular basis of inflammatory amplification, cellular aging, and immune response are critical to COPD pathogenesis. Our aim was to investigate EP300 (histone acetylase, HAT), HDAC 2 (histone deacetylase), HDAC3, and HDAC4 gene expression, telomere length, and differentiation ability to M1/M2 macrophages. For this investigation, 105 COPD patients, 42 smokers, and 73 non-smoker controls were evaluated. We identified a reduced HDAC2 expression in patients with mild, moderate, and severe severity; a reduced HDAC3 expression in patients with moderate and severe severity; an increased HDAC4 expression in patients with mild severity; and a reduced EP300 expression in patients with severe severity. Additionally, HDAC2 expression was reduced in patients with emphysema and exacerbator, along with a reduced HDAC3 expression in patients with emphysema. Surprisingly, smokers and all COPD patients showed telomere shortening. COPD patients showed a higher tendency toward M2 markers. Our data implicate genetic changes in COPD phenotypes and severity, in addition to M2 prevalence, that might influence future treatments and personalized therapies.
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Enfisema , Enfermedad Pulmonar Obstructiva Crónica , Enfisema Pulmonar , Humanos , Macrófagos , Senescencia Celular/genética , Expresión GénicaRESUMEN
INTRODUCTION: Although COPD patients commonly present respiratory complaints despite pharmacological treatment, dyspnea does not correlate directly and linearly with spirometric data, a fact that makes it difficult to select patients for pulmonary rehabilitation. Thus, seems logical that the measurement of respiratory muscle strength could help in this initial assessment if it presents a good correlation with exercise capacity. The aim of this study is to assess whether patients with muscle weakness, characterized as a reduction in maximal inspiratory pressure (PImax) below 70% of predicted value, have a good relationship between the assessed respiratory muscle strength and the exercise capacity measured by the 6-min walk test (6MWT) in patients with COPD. METHODS: Patients diagnosed with COPD according to the 2019 Global Initiative for Chronic Obstructive Lung Disease (GOLD) on regular use of their medications, without exacerbations for 3 months or more and with respiratory muscle weakness (PImax < 70% of predicted) performed 6MWT in a 30-m-long flat corridor. RESULTS: Data from 81 patients were analyzed. There was a strong correlation between the distance of the 6MWD with the PImax (r = 0.764, p < 0.0001). When separating the sample by the 350-m cut in the 6MWD, we found that the patients with the worst performance in the test are those who present the greatest respiratory muscle weakness. CONCLUSION: PImax correlates well with exercise capacity, and patients with respiratory muscle weakness could be referred to a pulmonary rehabilitation protocol tied to inspiratory muscle training.
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Tolerancia al Ejercicio , Enfermedad Pulmonar Obstructiva Crónica , Ejercicios Respiratorios , Prueba de Esfuerzo , Humanos , Debilidad Muscular/diagnóstico , Enfermedad Pulmonar Obstructiva Crónica/diagnóstico , Músculos RespiratoriosRESUMEN
BACKGROUND/OBJECTIVE: The aim of this study was to explore the associations between nailfold videocapillaroscopy (NVC) and pulmonary function tests (PFTs) in systemic sclerosis (SSc) patients. METHODS: This was a longitudinal study with follow-up of unselected Brazilian SSc patients. Baseline clinical examination, serological workup, high-resolution chest tomography, and NVC were performed. Pulmonary function test was performed at baseline and after 24 months. Pulmonary function test worsening over time was defined as either a ΔFVC decline ≥10% or a ΔFVC decline ≥5% and <9%, combined with a ΔDLCO decline ≥15%, at 24 months. The NVC parameters were quantitatively (capillary density number, dimension, morphology, and hemorrhages) and qualitatively (NVC pattern) evaluated according to the consented standardized definitions of the EULAR Study Group on Microcirculation in Rheumatic Diseases. RESULTS: Seventy-nine patients were included. Fifty-nine were rated to have a scleroderma pattern (6 "early"/16 "active"/37 "late"). The mean FVC and DLCO were 76.8% and 67.2% at baseline and 73.8% and 64.3% at 24 months, respectively. After multivariate analysis, we found that a reduced baseline FVC was associated with reduced capillary density (odds ratio [OR], 11; 95% confidence interval [CI], 1.5-90.7; p = 0.03) and a reduced baseline DLCO associated with the late scleroderma pattern (OR, 6.75; 95% CI, 1.09-42; p = 0.03). No association between worsening of PFT over time and NVC was found. CONCLUSIONS: The association between NVC and PFTs might corroborate the link between microangiopathy and interstitial lung disease in patients with SSc. This finding might strengthen the idea of incorporating NVC as a tool to predict progressive interstitial lung disease in these patients in the future.
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Enfermedades Pulmonares Intersticiales , Esclerodermia Sistémica , Capilares , Estudios de Seguimiento , Humanos , Estudios Longitudinales , Angioscopía Microscópica , Uñas , Pruebas de Función Respiratoria , Esclerodermia Sistémica/complicaciones , Esclerodermia Sistémica/diagnósticoRESUMEN
BACKGROUND: Systemic sclerosis (SSc) is a rare disease, and the presence of pulmonary hypertension can be a determining factor in prognosis. The aim of this study was to evaluate the diagnosis, profile, and prognosis of systemic sclerosis pulmonary hypertension (SSc-PH) diagnosed by systematic screening in a Brazilian population. METHODS: A cohort of SSc patients underwent systematic screening for SSc-PH. Patients were referred for right heart catheterization (RHC) according to transthoracic echocardiogram or a combination of diagnostic tools. The clinical, immunological, and hemodynamic features and prognosis after 3 years were evaluated. RESULTS: Twenty patients underwent RHC. SSc pulmonary arterial hypertension (SSc-PAH) was the most common group of SSc-PH. These patients had long disease duration, high urate levels and highly elevated mean pulmonary arterial pressure (mPAP) and peripheral vascular resistance (PVR) on hemodynamics. Patients with mPAP > 20- < 25 mmHg had hemodynamic features of intermediate disease. Patients with SSc-PH associated to interstitial lung disease (SSc-ILD-PH) had signs of vasculopathy on hemodynamics. In patients with no-SSc-PH, the survival at 1, 2, and 3 years was 96%, 92% and 92%, respectively and in patients with SSc-PH it was 86.7%, 60% and 53.3%, respectively. CONCLUSIONS: Patients identified with SSc-PAH and SSc-ILD-PH in our screening had severe clinical and hemodynamic features. Mortality remains high in SSc-PH but was more related to Bo-PAH and SSc-ILD-PH, while in SSc-PAH, the prognosis was better. TRIAL REGISTRATION: Current Controlled Trials ISRCTN 72968188, July 8th, 2021. Retrospectively registered.
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Hemodinámica , Hipertensión Pulmonar/diagnóstico , Enfermedades Pulmonares Intersticiales/fisiopatología , Hipertensión Arterial Pulmonar/diagnóstico , Esclerodermia Sistémica/fisiopatología , Adulto , Brasil , Cateterismo Cardíaco , Femenino , Estudios de Seguimiento , Humanos , Hipertensión Pulmonar/complicaciones , Hipertensión Pulmonar/fisiopatología , Enfermedades Pulmonares Intersticiales/complicaciones , Masculino , Persona de Mediana Edad , Pronóstico , Hipertensión Arterial Pulmonar/complicaciones , Hipertensión Arterial Pulmonar/fisiopatología , Estudios Retrospectivos , Esclerodermia Sistémica/complicaciones , Resistencia VascularRESUMEN
BACKGROUND: The role of lung ultrasound (LUS) in evaluating the mid- and long-term prognoses of patients with COVID-19 pneumonia is not yet known. The objectives of this study were to evaluate associations between LUS signs at the time of screening and clinical outcomes 1 month after LUS and to assess LUS signs at the time of presentation with known risk factors for COVID-19 pneumonia. METHODS: This was a retrospective study of data prospectively collected 1 month after LUS screening of 447 adult patients diagnosed with COVID-19 pneumonia. Sonographic examination was performed in screening tents with the participants seated. The LUS signs (B-lines > 2, coalescent B-lines, and subpleural consolidations) were captured in six areas of each hemithorax and a LUS aeration score was calculated; in addition, the categories of disease probability based on patterns of LUS findings (high-probability, intermediate-probability, alternate, and low-probability patterns) were evaluated. The LUS signs at patients' initial evaluation were related to the following outcomes: symptomatology, the need for hospitalization or invasive mechanical ventilation (IMV), and COVID-19-related death. RESULTS: According to the evaluations performed 1 month after LUS screening, 36 patients were hospitalised, eight of whom required intensive care unit (ICU) admission and three of whom died. The presence of coalescent B-lines was associated with the need for hospitalization (p = 0.008). The presence of subpleural consolidations was associated with dyspnoea (p < 0.0001), cough (p = 0.003), the need for hospitalization (p < 0.0001), the need for ICU admission (p < 0.0001), and death (p = 0.002). A higher aeration score was associated with dyspnoea (p < 0.0001), the need for hospitalization (p < 0.0001), the need for ICU admission (p < 0.0001), and death (p = 0.003). In addition, patients with a high-probability LUS pattern had a higher aeration score (p < 0.0001) and more dyspnoea (p = 0.024) and more often required hospitalization (p < 0.0001) and ICU admission (p = 0.031). CONCLUSIONS: In patients with COVID-19 pneumonia, LUS signs were related to respiratory symptoms 1 month after LUS screening. Strong relationships were identified between LUS signs and the need for hospitalization and death.
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OBJECTIVES: The aim of this study was to describe findings from lung ultrasound (LUS) and computed tomography (CT) in health professionals with coronavirus disease 2019 pneumonia and to evaluate the associations of the findings of both tests. METHODS: This cross-sectional observational study evaluated 45 health professionals who were initially seen in screening tents and had a diagnosis of coronavirus disease 2019 as confirmed by a reverse transcription polymerase chain reaction and lung involvement diagnosed by LUS. Subsequently, these individuals were admitted to the hospital, where chest CT was performed. Aeration scores were obtained for the LUS examinations based on the following findings: more than 2 B-lines, coalescent B-lines, and subpleural consolidations. A subjective assessment of the extent of lung disease on CT was performed on the basis of the percentage of lung parenchyma involvement as follows: 25% or less, 25% to 50%, and greater than 50%. RESULTS: Regarding LUS signs, more than 2 B-lines, coalescent B-lines, and subpleural consolidations were present in 73.3%, 68.2%, and 24.4% of cases, respectively. The main findings on CT were ground glass opacities, a crazy-paving pattern, and consolidations (66.7%, 20%, and 20% of cases); 17.8% of cases had examinations without abnormalities. Patients with more than 2 B-lines on LUS had more ground glass opacity areas on CT (P = .0007), whereas patients with subpleural consolidations on LUS had more consolidations on CT (P < .0001). In addition, patients with higher LUS aeration scores had more extensive disease on CT (P < .0001). CONCLUSIONS: Lung ultrasound can detect lung injury even in the presence of normal CT results. There are associations between the abnormalities detected by both methods, and a relationship also exists between LUS aeration scores and the disease extent on CT.
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COVID-19 , Estudios Transversales , Humanos , Pulmón/diagnóstico por imagen , SARS-CoV-2 , Tomografía Computarizada por Rayos X , UltrasonografíaRESUMEN
PURPOSE: To evaluate ultrasound signs of coronavirus disease-19 (COVID-19) pneumonia in symptomatic healthcare professionals and to correlate those changes with clinical findings. METHODS: All patients underwent real-time polymerase chain reaction (RT-PCR), lung ultrasound (LUS) and clinical evaluation on the same day. In each of the 12 areas evaluated in the LUS, the LUS signs were scored to generate the aeration score. RESULTS: A total of 409 participants had positive PCR, with a median age of 41 (35-51) years. All participants had clinical symptoms, with cough in 84.1%, fever in 69.7%, and dyspnea in 36.2% of cases. In the LUS, 72.6% of participants had B-lines >2, 36.2% had coalescent B-lines, and 8.06% had subpleural consolidations. The median aeration score was 3 (2-7). The aeration score differed significantly regarding the presence of cough (P = .002), fever (P = .001), and dyspnea (P < .0001). The finding of subpleural consolidations in the LUS showed significant differences between participants with or without dyspnea (P < .0001). CONCLUSIONS: In healthcare professionals with COVID-19, LUS plays a key role in the characterization of lung involvement. Although B-lines are the most common ultrasound sign, subpleural consolidations are those that most impact the respiratory condition.
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Infecciones por Coronavirus/diagnóstico por imagen , Personal de Salud , Pulmón/diagnóstico por imagen , Neumonía Viral/diagnóstico por imagen , Adulto , Betacoronavirus/genética , Betacoronavirus/aislamiento & purificación , COVID-19 , Prueba de COVID-19 , Vacunas contra la COVID-19 , Técnicas de Laboratorio Clínico/métodos , Infecciones por Coronavirus/diagnóstico , Infecciones por Coronavirus/patología , Infecciones por Coronavirus/virología , Estudios Transversales , Femenino , Humanos , Pulmón/patología , Masculino , Persona de Mediana Edad , Pandemias , Neumonía Viral/diagnóstico , Neumonía Viral/patología , Neumonía Viral/virología , Reacción en Cadena en Tiempo Real de la Polimerasa , SARS-CoV-2 , Ultrasonografía/métodosRESUMEN
Purpose: We aimed to correlate three polymorphisms of the Hedgehog Interacting Protein (HHIP) gene with the three main phenotypes of the chronic obstructive pulmonary disease (frequent exacerbator (FE), asthma/COPD overlap (ACO), and emphysema with hyperinflation). Patients and methods: A cross-sectional study was carried out in the Department of Pulmonology at the Rio de Janeiro State University from February 2015 to July 2018. A total of 81 patients diagnosed with COPD according to the criteria of the Global Initiative for Chronic Obstructive Lung Disease (GOLD) were enrolled. The subjects were divided into three distinct groups according to their phenotypes (FE, ACO and emphysema-hyperinflation). Three polymorphisms of the HHIP gene that are often reported as allegedly involved in the pathogenesis of COPD were analysed: rs1828591, rs13118928, and rs6537296. Real-time PCR - TAQMAN SNP Genotyping Assay was performed. The statistical analysis was carried out with the SPSS program with a multivariate analysis with a 95% confidence interval. Results: An increase in the frequency of the A allele of the rs13118928 HHIP gene polymorphism was observed in the group of subjects with COPD and emphysema-hyperinflation phenotype when compared with those in the FE phenotype (p=0.019) and subjects with ACO (p=0.04). However, the subjects with emphysema-hyperinflation phenotype presented more often the A allele (p=0.04). The genotypic analysis confirmed the difference between the emphysema-hyperinflation and ACO phenotypes, with a higher prevalence of the AA genotype in the emphysema-hyperinflation group (p=0.04). The ACO and FE phenotype subjects showed no difference in these polymorphisms. No difference was found in the frequency of the polymorphisms rs1828591 (p= 0.552) and rs6537296 (p=0.296) in the three phenotypes evaluated. Conclusion: The presence of the A allele in the rs13118928 polymorphism of the HHIP gene may be related to the emphysema-hyperinflation phenotype.
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Proteínas Portadoras/genética , Glicoproteínas de Membrana/genética , Polimorfismo Genético , Enfermedad Pulmonar Obstructiva Crónica/genética , Anciano , Asma/genética , Estudios Transversales , Femenino , Humanos , Masculino , Persona de Mediana Edad , Fenotipo , Enfisema Pulmonar/genéticaRESUMEN
PURPOSE: Activity/remission differentiation is a great challenge in the follow-up and treatment of sarcoidosis patients. Angiotensin-converting enzyme (ACE) and high sensitivity C-reactive protein (hs-CRP) were proposed as sarcoidosis biomarkers. More recently, chitotriosidase (CHITO) has been described as a better alternative. This study has the aim to evaluate the association of CHITO activity, ACE, hs-CRP or a combination of these biomarkers and to construct a clinical algorithm to differentiate between sarcoidosis activity/remission status. METHODS: Forty-six patients with either active sarcoidosis or sarcoidosis in remission and 21 healthy individuals were included. ACE, hs-CRP, and CHITO were evaluated in serum samples. Comparisons of the laboratory variable means among groups were performed by linear models. The cutoff points of the biomarkers for activity/remission differentiation were calculated using the Youden's index. Biomarker cutoff points and decision tree classifier (DTC) performance were estimated by their leave-one-out cross-validation (LOOCV) accuracy (Acc), sensitivity (Se), and specificity (Sp). RESULTS: A 55% mean Se and a 100% mean Sp were found for CHITO, while an 88% Se and a 47% Sp were found for ACE, and a 66% Se and a 68% Sp for hs-CRP cutoff points for activity/remission differentiation. The DTC algorithm with CHITO, hs-CRP, and ACE information had an LOOCV mean Acc of 82%, Se of 78%, and Sp of 89% for sarcoidosis activity/remission differentiation. CONCLUSIONS: The algorithm involving CHITO, hs-CRP, and ACE could be a suitable strategy for differentiation between sarcoidosis activity/remission status.
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Proteína C-Reactiva/metabolismo , Hexosaminidasas/metabolismo , Peptidil-Dipeptidasa A/metabolismo , Sarcoidosis/metabolismo , Adulto , Anciano , Estudios de Casos y Controles , Femenino , Humanos , Masculino , Persona de Mediana Edad , Inducción de Remisión , Índice de Severidad de la Enfermedad , Adulto JovenRESUMEN
OBJECTIVE: Alpha-1-antitrypsin deficiency is a relatively prevalent, but under-diagnosed, genetic disease. The objective of this study was to assess whether the systematic screening for alpha-1-antitrypsin deficiency in all patients with chronic obstructive pulmonary disease from a tertiary service has an impact on the number of patients being diagnosed with this condition. RESULTS: Chronic obstructive pulmonary disease patients were screened for alpha-1-antitrypsin deficiency using immunonephelometry. The presence of a mutation was confirmed by molecular study of the SERPINA1 gene or by genetic sequencing, as needed. A total of 551 patients with chronic obstructive pulmonary disease were analyzed. Among these, 40 (7.2%) had some genetic mutation, while 11 (2%) had a Pi*ZZ genotype, resulting in severe respiratory illness. The systematic evaluation of chronic obstructive pulmonary disease patients revealed that screening is an effective method to diagnose alpha-1-antitrypsin deficiency. Early diagnosis may facilitate smoking cessation and initiation of treatment to maintain lung function.
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Enfermedad Pulmonar Obstructiva Crónica/diagnóstico , Deficiencia de alfa 1-Antitripsina/diagnóstico , alfa 1-Antitripsina/análisis , Anciano , Brasil/epidemiología , Femenino , Humanos , Inmunoturbidimetría , Masculino , Persona de Mediana Edad , Mutación , Enfermedad Pulmonar Obstructiva Crónica/epidemiología , Enfermedad Pulmonar Obstructiva Crónica/genética , Centros de Atención Terciaria , alfa 1-Antitripsina/genética , Deficiencia de alfa 1-Antitripsina/epidemiología , Deficiencia de alfa 1-Antitripsina/genéticaRESUMEN
OBJECTIVE: To investigate the effect of rituximab on microcirculation in long-term SSc. RESULTS: Four patients with diffuse SSc over 3 years of disease received rituximab cycles of two 1-g infusions every 6 months for 2 years. Videocapillaroscopy was performed at baseline, 12 months, and 24 months and semi-quantitative scoring of videocapillaroscopy abnormalities was performed and the microangiopathy evolution score (MES: range 0-9) was calculated. The mean disease duration was 5 years (range 3-15). On videocapillaroscopy, giant capillaries and hemorrhages remained stable from baseline to 24 months. Capillary loss, abnormally-shaped capillaries, and MES stabilized at 12 months and increased by 24.5% and 28% at 24 months. Rituximab improves microcirculation in long-term SSc. Stabilization and reduced progression of microcirculation abnormalities were achieved at 12 and 24 months, respectively.
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Microcirculación , Rituximab/uso terapéutico , Esclerodermia Sistémica/sangre , Esclerodermia Sistémica/tratamiento farmacológico , Adulto , Femenino , Humanos , Masculino , Microcirculación/efectos de los fármacos , Angioscopía Microscópica , Persona de Mediana Edad , Pruebas de Función Respiratoria , Rituximab/farmacología , Esclerodermia Sistémica/fisiopatología , Factores de TiempoRESUMEN
The INHALATOR study was a randomized, multicentre, open label, two-period of 7 days each, crossover study, with 7 days of washout in-between, aiming to evaluate the correct use, satisfaction and preference between Breezhaler® and Respimat® devices in patients under daily use of open Spiriva® or open Onbrize®, as monotherapy for treatment of mild or moderate COPD. Patients aged ≥40 years with a smoking history of at least 10 pack-year were included in the study. Primary endpoint was the rate of correct use of each device at the first day of treatment after reading the drug leaflet information and was evaluated under the supervision of a trained evaluator. At the end of each treatment phase, the inhaler use was re-evaluated and a satisfaction questionnaire was completed. The patients' preference for the inhaler devices was assessed at the end of the study. After exclusions due to screening failures, 140 patients were randomized: 136 received at least one dose of Breezhaler® and 135 of Respimat®. At treatment start, the rate of correct inhaler use was 40.4% (95%CI: 32.2%-48.7%) for Breezhaler® and 36.3% (95%CI: 28.2%-44.4%) for Respimat® (pâ¯=â¯0.451). After 7 days, the rates were 68.9% (95%CI: 61.1%-76.7%) and 60.4% (95%CI: 52.2%-68.7%), respectively (pâ¯=â¯0.077). According to the Feeling of Satisfaction with Inhaler Questionnaire - FSI 10 patients were more satisfied using Breezhaler® than Respimat® and 57.1% preferred using Breezhaler® (pâ¯=â¯0.001) while 30.1% preferred Respimat® (pâ¯<â¯0.001).
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Nebulizadores y Vaporizadores , Prioridad del Paciente , Satisfacción del Paciente , Enfermedad Pulmonar Obstructiva Crónica/tratamiento farmacológico , Enfermedad Pulmonar Obstructiva Crónica/psicología , Administración por Inhalación , Adulto , Anciano , Estudios Cruzados , Femenino , Humanos , Masculino , Persona de Mediana Edad , Encuestas y CuestionariosRESUMEN
BACKGROUND: Burkholderia cepacia complex is a group of opportunistic pathogens in cystic fibrosis (CF) patients believed to be associated with poor prognosis and patient-to-patient transmissibility. Little is known about clinical outcomes after B. vietnamiensis chronic colonization/infection. CASE PRESENTATION: A 33 yo male patient had diagnosis of CF by 7 yo, after recurrent pneumonia during infancy and lobectomy (left upper lobe) at 6 yo. Burkholderia cepacia complex (Bcc) was first isolated by 13 yo, and the patient fulfilled the criteria for chronic colonization by 15 yo. In the following 16 years (1997-2013), there was intermittent isolation of P. aeruginosa and continuous isolation of Bcc, identified as B. vietnamiensis. There was clinical and laboratorial stability for 16 years with annual rate of decline in forced expiratory volume in 1 s (FEV1) and forced vital capacity (FVC) of 1.61 and 1.35%, respectively. From 2013 to 2015, there was significant clinical and lung function deterioration: annual rate of decline in FEV1 and FVC was 3 and 4.1%, respectively while body mass index decreased from 18.1 to 17.1. Episodes of hemoptysis and respiratory exacerbations (with hospital admissions) became more frequent. CF related diabetes was diagnosed (fasting glycemia: 116 mg/dL, oral glucose tolerance test: 305 mg/dL). Because of the severity of the disease in the last years, in addition to traditional microbiological surveillance, microbiome analysis by next generation sequencing (NGS) was performed on respiratory secretions. The NGS showed that 97% of the sequencing data were attributed to genus Burkholderia. CONCLUSIONS: We report the case of a 33-year-old male CF patient known to have chronic infection with B. vietnamiensis who remained clinically stable for 16 years and presented recent clinical and laboratorial deterioration. Microbiome analysis of respiratory secretions was performed in 3 samples collected in 2014-2015. Clinical deterioration overlapped with cystic fibrosis-related diabetes and microbiome composition revealed no significant differences when compared microbiome results to culture dependent methods.
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Complejo Burkholderia cepacia/aislamiento & purificación , Portador Sano/microbiología , Fibrosis Quística/microbiología , Fibrosis Quística/fisiopatología , Sistema Respiratorio/microbiología , Adolescente , Adulto , Brasil , Niño , Fibrosis Quística/complicaciones , Diabetes Mellitus/etiología , Progresión de la Enfermedad , Volumen Espiratorio Forzado , Humanos , Masculino , Microbiota , Pseudomonas aeruginosa/aislamiento & purificación , Capacidad Vital , Adulto JovenRESUMEN
BACKGROUND: Pulmonary deterioration after B.cepacia complex (BCC) colonization has a heterogeneous pattern. The aim was to investigate the clinical outcome of BCC colonization in CF patients chronically colonized with P. aeruginosa. METHODS: CF patients chronically colonized with P. aeruginosa were divided into three groups: intermittent (I), chronic (II) and no colonization (III) with BCC. Body mass index (BMI) percentile and spirometric parameters were analyzed at three different times in each group. RESULTS: Fifty-six patients chronically colonized with P. aeruginosa were included. Of these, 27 also had evidence of BCC colonization (13 intermittent and 14 chronic). BMI percentile was significantly lower among patients chronically colonized by both P. aeruginosa and BCC. Mean values of FEV1 and FVC % were also significantly lower in these patients, both at the time of chronic BCC colonization and 24 months forward. CONCLUSIONS: Chronic BCC colonization is associated with significant loss of lung function. Lower BMI might be a risk factor for chronic BCC colonization, preceding these events.
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Infecciones por Burkholderia/fisiopatología , Complejo Burkholderia cepacia , Portador Sano/fisiopatología , Fibrosis Quística/fisiopatología , Índice de Masa Corporal , Estudios de Casos y Controles , Niño , Coinfección , Progresión de la Enfermedad , Femenino , Volumen Espiratorio Forzado , Humanos , Estudios Longitudinales , Masculino , Infecciones por Pseudomonas/fisiopatología , Pseudomonas aeruginosa , Estudios Retrospectivos , Espirometría , Capacidad VitalRESUMEN
INTRODUCTION: The Sleep Apnea Clinical Score (SACS) and the Berlin Questionnaire (BQ) are used to predict the likelihood of obstructive sleep apnea (OSA). The Epworth Sleepiness Scale (ESS) is used to assess daytime sleepiness, a common OSA symptom. These clinical tools help prioritize individuals with the most severe illness regarding on whom polysomnography (PSG) should be performed. It is necessary to check the applicability of these tools in patients with chronic obstructive pulmonary disease (COPD). The aim of this study is to compare SACS, BQ, and ESS performance in patients with COPD. METHODS: The SACS, BQ, and ESS were applied to 91 patients with COPD. From this group, 24 underwent PSG. In this transversal study, these three tests were compared regarding their likelihood to predict OSA in patients with COPD using receiver-operating characteristic curve statistics. RESULTS: In this sample, 58 (63.7%) patients were men, and their mean age was 69.4±9.6 years. Fourteen patients (15.4%) had a high probability of OSA by SACS, 32 (32.5%) had a high probability by BQ, and 37 (40.7%) had excessive diurnal somnolence according to the ESS. From the 24 patients who underwent PSG, OSA diagnosis was confirmed in five (20.8%), according to the American Academy of Sleep Medicine criteria. BQ and ESS did not accurately predict OSA in this group of patients with COPD, with a receiver-operating characteristic curve area under the curves of 0.54 (95% CI: 0.329-0.745, P=0.75) and 0.69 (95% CI: 0.47-0.860, P=0.10), respectively. SACS performance was significantly better, with an area under the curve of 0.82 (95% CI: 0.606-0.943, P=0.02). CONCLUSION: SACS was better than BQ and ESS in predicting OSA in this group of patients with COPD.
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INTRODUCTION: Diagnostic approaches to patients with a pleural effusion must be precise because many procedures depend on the nature of the fluid in the effusion. To date, no biochemical test is considered an appropriate alternative to Light's criteria. This study compared the absolute pleural cholesterol (PC) level and the pleural cholesterol/serum cholesterol (PC/SC) ratio with Light's criteria to determine exudative pleural effusions. MATERIALS AND METHODOLOGY: This study was a case series of 100 consecutive patients with pleural effusions. The clinical parameters that were used to diagnosis an exudative effusion included the cholesterol level, a pleural cholesterol level ≥ 50 mg/dL, a pleural/serum ratio ≥ 0.4, and Light's criteria. The sensitivity, specificity, and positive and negative predictive values of each test for the diagnosis of an exudative effusion were assessed. RESULTS: A total of 79 patients were definitively diagnosed with an exudative effusion and were included in the trial and analyzed. The mean PC level in the exudates was 90.39 mg/dL. The PC levels demonstrated a sensitivity of 97.22%, a specificity of 85.71%, a positive predictive value of 98.59% and a negative predictive value of 75%. The PC/SC ratio demonstrated a sensitivity of 81.48%, a specificity of 57.14%, a positive predictive value of 93.61% and a negative predictive value of 28.57%. CONCLUSION: The pleural cholesterol dosage level and the pleural/serum cholesterol ratio can be utilized as unique biomarkers to identify an exudative effusion and replace Light's criteria.
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Sarcoidosis is a multisystem granulomatous disorder of unknown etiology. Hereditary etiology has been proposed as a cause of the sarcoidosis, and some Human Leucocyte Antigen (HLA) alleles have been related to the diagnosis or severity of the disease. Löfgren's syndrome has been linked to patients with the DRB1*03 allele, and non-resolving disease has been associated with the DRB1*07, DRB1*14 and DRB1*15 alleles. However, the results observed in Caucasian patients are not reproducible in other populations, such as in Japanese individuals. The aim of this study is to examine the HLA alleles in Brazilian patients with sarcoidosis confirmed by biopsy. Sixty-three patients were included in the study, and the HLA alleles were compared with 126 control individuals. HLA-A, -B, -C, -DRB1 and -DQB1 genes were typed using a Luminex Multi-analyte profiling system (One Lambda, Inc. Canoga Park, CA). Among sarcoidosis patients, the HLA A*23, A*80, B*08, B*41, DQB1*05 and DRB1*14 antigens tended to be more common than in the controls, whereas the B*44, B*45, B*51, B*58, DRB1*15 and DRB1*16 alleles were more frequently found in control subjects than in the sarcoidosis patients. However, after Bonferroni correction, only the HLA-DRB1*14 allele was found to be significantly different between sarcoidosis patients and controls (pC=0.0047, OR=11.69, CI=2.47-55.22). This allele was more frequent in mestizos and black patients. The presence of an HLA-DRB1*14 allele might determine the risk of sarcoidosis in Brazilian individuals, especially in mestizos and black patients.
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Antígenos HLA/genética , Sarcoidosis/genética , Adulto , Alelos , Brasil , Estudios de Cohortes , Etnicidad/genética , Femenino , Frecuencia de los Genes , Antígenos HLA/inmunología , Prueba de Histocompatibilidad , Humanos , Desequilibrio de Ligamiento , Masculino , Persona de Mediana Edad , Sarcoidosis/diagnóstico , Sarcoidosis/inmunologíaRESUMEN
OBJECTIVE: To analyze the epidemiological characteristics of sarcoidosis patients in the city of Rio de Janeiro, Brazil. METHODS: A descriptive, case-control study involving 100 sarcoidosis patients under outpatient treatment between 2008 and 2010 at the Pedro Ernesto University Hospital, located in the city of Rio de Janeiro, Brazil. The diagnosis of sarcoidosis was based on clinical, radiological, biochemical, and histopathological criteria. RESULTS: There was a predominance of females in the 35-40 year age bracket (range, 7-69 years), who accounted for 65% of the sample, although there was a second peak at approximately 55 years of age. The most common symptom was dyspnea (in 47%), and the most common radiological findings were pulmonary and lymph node involvement (stage II; in 43%), followed by stage III (in 20%), stage I (in 19%), stage 0 (in 15%), and stage IV (in 3%). No pleural effusion or digital clubbing was observed at diagnosis. The tuberculin skin test was negative in 94 patients. Spirometric findings at diagnosis were normal in 61 patients; indicative of obstructive lung disease in 21; and indicative of restrictive lung disease in 18. The most common biopsy sites were the lungs (principally by bronchoscopy) and the skin, the diagnosis being confirmed by biopsy in 56% and 29% of the cases, respectively. Treatment with prednisone was initiated in 75% of the patients and maintained for more than 2 years in 19.7%. CONCLUSIONS: This study corroborates the findings of previous studies regarding the epidemiological characteristics of sarcoidosis patients.