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Antiasmáticos , Anticorpos Monoclonais Humanizados , Asma , Interleucina-5 , Adulto , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Antiasmáticos/uso terapêutico , Anticorpos Monoclonais Humanizados/uso terapêutico , Asma/tratamento farmacológico , Eosinofilia/tratamento farmacológico , Interleucina-5/antagonistas & inibidores , Receptores de Interleucina-5 , Índice de Gravidade de DoençaRESUMO
BACKGROUND AND OBJECTIVE: EpiGETIF is a web-based, multicentre clinical database created in 2019 aiming for prospective collection of data regarding therapeutic rigid bronchoscopy (TB) for malignant central airway obstruction (MCAO). METHODS: Patients were enrolled into the registry from January 2019 to November 2022. Data were prospectively entered through a web-interface, using standardized definitions for each item. The objective of this first extraction of data was to describe the population and the techniques used among the included centres to target, facilitate and encourage further studies in TB. RESULTS: Overall, 2118 patients from 36 centres were included. Patients were on average 63.7 years old, mostly male and smokers. Most patients had a WHO score ≤2 (70.2%) and 39.6% required preoperative oxygen support, including mechanical ventilation in 6.7%. 62.4% had an already known histologic diagnosis but only 46.3% had received any oncologic treatment. Most tumours were bronchogenic (60.6%), causing mainly intrinsic or mixed obstruction (43.3% and 41.5%, respectively). Mechanical debulking was the most frequent technique (67.3%), while laser (9.8%) and cryo-recanalization (2.7%) use depended on local expertise. Stenting was required in 54.7%, silicone being the main type of stent used (55.3%). 96.3% of procedure results were considered at least partially successful, resulting in a mean 4.1 points decrease on the Borg scale of dyspnoea. Complications were noted in 10.9%. CONCLUSION: This study exposes a high volume of TB that could represent a good source of future studies given the dismal amount of data about the effects of TB in certain populations and situations.
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Obstrução das Vias Respiratórias , Broncoscopia , Sistema de Registros , Humanos , Broncoscopia/métodos , Masculino , Obstrução das Vias Respiratórias/diagnóstico , Obstrução das Vias Respiratórias/terapia , Obstrução das Vias Respiratórias/etiologia , Pessoa de Meia-Idade , Feminino , Estudos Prospectivos , Idoso , Stents , Neoplasias Pulmonares/complicaçõesRESUMO
BACKGROUND: One of the major challenges in managing allergic bronchopulmonary aspergillosis remains consistent and reproducible assessment of response to treatment. RESEARCH QUESTION: What are the most relevant changes in CT scan parameters over time for assessing response to treatment? STUDY DESIGN AND METHODS: In this ancillary study of a randomized clinical trial (NebuLamB), patients with asthma with available CT scan and without exacerbation during a 4-month allergic bronchopulmonary aspergillosis exacerbation treatment period (corticosteroids and itraconazole) were included. Changed CT scan parameters were assessed by systematic analyses of CT scan findings at initiation and end of treatment. CT scans were assessed by two radiologists anonymized to the clinical data. Radiologic parameters were determined by selecting those showing significant changes over time. Improvement of at least one, without worsening of the others, defined the radiologic response. Agreement between radiologic changes and clinical and immunologic responses was likewise investigated. RESULTS: Among the 139 originally randomized patients, 132 were included. We identified five CT scan parameters showing significant changes at end of treatment: mucoid impaction extent, mucoid impaction density, centrilobular micronodules, consolidation/ground-glass opacities, and bronchial wall thickening (P < .05). These changes were only weakly associated with one another, except for mucoid impaction extent and density. No agreement was observed between clinical, immunologic, and radiologic responses, assessed as an overall response, or considering each of the parameters (Cohen κ, -0.01 to 0.24). INTERPRETATION: Changes in extent and density of mucoid impaction, centrilobular micronodules, consolidation/ground-glass opacities, and thickening of the bronchial walls were found to be the most relevant CT scan parameters to assess radiologic response to treatment. A clinical, immunologic, and radiologic multidimensional approach should be adopted to assess outcomes, probably with a composite definition of response to treatment. TRIAL REGISTRATION: ClinicalTrials.gov; No.: NCT02273661; URL: www. CLINICALTRIALS: gov).
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Antifúngicos , Aspergilose Broncopulmonar Alérgica , Asma , Itraconazol , Tomografia Computadorizada por Raios X , Humanos , Aspergilose Broncopulmonar Alérgica/diagnóstico por imagem , Aspergilose Broncopulmonar Alérgica/tratamento farmacológico , Masculino , Feminino , Tomografia Computadorizada por Raios X/métodos , Asma/diagnóstico por imagem , Asma/tratamento farmacológico , Adulto , Pessoa de Meia-Idade , Itraconazol/uso terapêutico , Antifúngicos/uso terapêutico , Resultado do Tratamento , Corticosteroides/uso terapêuticoAssuntos
Obstrução das Vias Respiratórias , Broncoscopia , Insuficiência Respiratória , Humanos , Broncoscopia/métodos , Obstrução das Vias Respiratórias/etiologia , Obstrução das Vias Respiratórias/diagnóstico , Obstrução das Vias Respiratórias/terapia , Insuficiência Respiratória/etiologia , Insuficiência Respiratória/terapia , Masculino , Feminino , Pessoa de Meia-Idade , Idoso , Neoplasias Pulmonares/complicações , Doença Aguda , Resultado do Tratamento , Estudos RetrospectivosRESUMO
INTRODUCTION: Primary spontaneous pneumothorax (PSP) is the presence of air in the pleural space, occurring in the absence of trauma and known lung disease. Standardized expert guidelines on PSP are needed due to the variety of diagnostic methods, therapeutic strategies and medical and surgical disciplines involved in its management. METHODS: Literature review, analysis of the literature according to the GRADE (Grading of Recommendation, Assessment, Development and Evaluation) methodology; proposals for guidelines rated by experts, patients and organizers to reach a consensus. Only expert opinions with strong agreement were selected. RESULTS: A large PSP is defined as presence of a visible rim along the entire axillary line between the lung margin and the chest wall and ≥ 2 cm at the hilum level on frontal chest X-ray. The therapeutic strategy depends on the clinical presentation: emergency needle aspiration for tension PSP; in the absence of signs of severity: conservative management (small PSP), needle aspiration or chest tube drainage (large PSP). Outpatient treatment is possible if a dedicated outpatient care system is previously organized. Indications, surgical procedures and perioperative analgesia are detailed. Associated measures, including smoking cessation, are described. CONCLUSION: These guidelines are a step towards PSP treatment and follow-up strategy optimization in France.
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Primary spontaneous pneumothorax (PSP) is the presence of air in the pleural space, occurring in the absence of trauma and known lung disease. Standardized expert guidelines on PSP are needed due to the variety of diagnostic methods, therapeutic strategies and medical and surgical disciplines involved in its management. Literature review, analysis of literature according to the GRADE (Grading of Recommendation Assessment, Development and Evaluation) methodology; proposals for guidelines rated by experts, patients, and organizers to reach a consensus. Only expert opinions with strong agreement were selected. A large PSP is defined as presence of a visible rim along the entire axillary line between the lung margin and the chest wall and ≥2 cm at the hilum level on frontal chest x-ray. The therapeutic strategy depends on the clinical presentation: emergency needle aspiration for tension PSP; in the absence of signs of severity: conservative management (small PSP), needle aspiration or chest tube drainage (large PSP). Outpatient treatment is possible if a dedicated outpatient care system is previously organized. Indications, surgical procedures and perioperative analgesia are detailed. Associated measures, including smoking cessation, are described. These guidelines are a step towards PSP treatment and follow-up strategy optimization in France.
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Humanos , Adulto , Pneumotórax/diagnóstico por imagem , Administração dos Cuidados ao Paciente/normas , Pneumotórax/terapia , Tomografia Computadorizada por Raios XRESUMO
BACKGROUND: A written action plan (WAP) for managing asthma exacerbations is recommended. OBJECTIVE: We aimed to compare the effect on unscheduled medical contacts (UMCs) of a digital action plan (DAP) accessed via a smartphone web app combined with a WAP on paper versus that of the same WAP alone. METHODS: This randomized, unblinded, multicenter (offline recruitment in private offices and public hospitals), and parallel-group trial included children (aged 6-12 years) or adults (aged 18-60 years) with asthma who had experienced at least 1 severe exacerbation in the previous year. They were randomized to a WAP or DAP+WAP group in a 1:1 ratio. The DAP (fully automated) provided treatment advice according to the severity and previous pharmacotherapy of the exacerbation. The DAP was an algorithm that recorded 3 to 9 clinical descriptors. In the app, the participant first assessed the severity of their current symptoms on a 10-point scale and then entered the symptom descriptors. Before the trial, the wordings and ordering of these descriptors were validated by 50 parents of children with asthma and 50 adults with asthma; the app was not modified during the trial. Participants were interviewed at 3, 6, 9, and 12 months to record exacerbations, UMCs, and WAP and DAP use, including the subjective evaluation (availability and usefulness) of the action plans, by a research nurse. RESULTS: Overall, 280 participants were randomized, of whom 33 (11.8%) were excluded because of the absence of follow-up data after randomization, leaving 247 (88.2%) participants (children: n=93, 37.7%; adults: n=154, 62.3%). The WAP group had 49.8% (123/247) of participants (children: n=45, 36.6%; mean age 8.3, SD 2.0 years; adults: n=78, 63.4%; mean age 36.3, SD 12.7 years), and the DAP+WAP group had 50.2% (124/247) of participants (children: n=48, 38.7%; mean age 9.0, SD 1.9 years; adults: n=76, 61.3%; mean age 34.5, SD 11.3 years). Overall, the annual severe exacerbation rate was 0.53 and not different between the 2 groups of participants. The mean number of UMCs per year was 0.31 (SD 0.62) in the WAP group and 0.37 (SD 0.82) in the DAP+WAP group (mean difference 0.06, 95% CI -0.12 to 0.24; P=.82). Use per patient with at least 1 moderate or severe exacerbation was higher for the WAP (33/65, 51% vs 15/63, 24% for the DAP; P=.002). Thus, participants were more likely to use the WAP than the DAP despite the nonsignificant difference between the action plans in the subjective evaluation. Median symptom severity of the self-evaluated exacerbation was 4 out of 10 and not significantly different from the symptom severity assessed by the app. CONCLUSIONS: The DAP was used less often than the WAP and did not decrease the number of UMCs compared with the WAP alone. TRIAL REGISTRATION: ClinicalTrials.gov NCT02869958; https://clinicaltrials.gov/ct2/show/NCT02869958.
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Antiasmáticos , Asma , Aplicativos Móveis , Adulto , Criança , Humanos , Asma/tratamento farmacológico , Autocuidado , Redação , Progressão da Doença , Antiasmáticos/uso terapêuticoRESUMO
INTRODUCTION: Primary spontaneous pneumothorax (PSP) is the presence of air in the pleural space, occurring in the absence of trauma and known lung disease. Standardized expert guidelines on PSP are needed due to the variety of diagnostic methods, therapeutic strategies and medical and surgical disciplines involved in its management. METHODS: Literature review, analysis of literature according to the GRADE (Grading of Recommendation Assessment, Development and Evaluation) methodology; proposals for guidelines rated by experts, patients, and organizers to reach a consensus. Only expert opinions with strong agreement were selected. RESULTS: A large PSP is defined as presence of a visible rim along the entire axillary line between the lung margin and the chest wall and ≥2 cm at the hilum level on frontal chest x-ray. The therapeutic strategy depends on the clinical presentation: emergency needle aspiration for tension PSP; in the absence of signs of severity: conservative management (small PSP), needle aspiration or chest tube drainage (large PSP). Outpatient treatment is possible if a dedicated outpatient care system is previously organized. Indications, surgical procedures and perioperative analgesia are detailed. Associated measures, including smoking cessation, are described. CONCLUSION: These guidelines are a step towards PSP treatment and follow-up strategy optimization in France.
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Anestesia , Medicina de Emergência , Pneumotórax , Transtornos Respiratórios , Humanos , Pneumotórax/terapia , Pneumotórax/cirurgia , Cuidados CríticosRESUMO
BACKGROUND: Obstructive sleep apnoea syndrome (OSAS) and severe asthma are frequently associated. This article focuses on the relationship between severe asthma phenotypes and OSAS. METHODS: FASE-CPHG was an observational, cross-sectional, prospective, multicentric study conducted in 104 non-academic hospitals from May-16 to July-17. 1465 patients with severe asthma were analysed and 1424 patients phenotyped. Clusters were compared for OSAS presence; independent factors associated with OSAS were identified by logistic regression. RESULTS: 11% of patients with severe asthma reported OSAS. OSAS incidence differed according to asthma phenotypes. 98% of OSAS patients belonged to the "obese asthma" cluster, and none to the "early onset allergic asthma" cluster. Independent factors associated with OSAS were obesity (OR=5.782 [3.927-8.512]), male gender (OR=3.047 [2.059-4.510]), high blood pressure (OR=2.875 [1.978-4.181]), depression (OR=2.552 [1.607-4.050]), late-onset asthma (OR=1.789 [1.167-2.743]) and atopy (OR=0.622 [0.408-0.948]). Moreover, OSAS patients were more frequently treated with long-term oral corticosteroids (30% vs 15%, p < 0.0001), that may contribute to the high prevalence of obesity in this group of patients. They were more frequently uncontrolled (78% vs 69%, p = 0.03) and they engaged in low level physical activity (vs 80% vs 68% p ≤ 0.001). CONCLUSION: Our study gives an innovative insight into OSAS associated with severe asthma. Most of patients with OSAS belonged to the cluster "obese asthma" and none to the cluster "early onset allergic asthma". In addition to male gender, arterial hypertension and depression, obese asthma, late onset asthma and non-atopic status were identified as specific risk factors. Oral corticosteroids seems to play a deleterious role. Phenotyping asthma can help physicians target severe asthmatic patients with OSAS and may avoid unnecessary examinations.
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Asma , Hipertensão , Apneia Obstrutiva do Sono , Humanos , Masculino , Corticosteroides , Asma/diagnóstico , Asma/epidemiologia , Estudos Transversais , Hipertensão/epidemiologia , Obesidade/complicações , Obesidade/epidemiologia , Estudos Prospectivos , Apneia Obstrutiva do Sono/diagnóstico , Apneia Obstrutiva do Sono/epidemiologia , Apneia Obstrutiva do Sono/terapiaRESUMO
BACKGROUND: Mineralogical analyses of bronchoalveolar lavage (BAL) may help in assessing past exposure to mineral particles. However, their interpretation relies on their quality, meaning their representativeness of the alveolar compartment. The aim of this study was to find predictive factors of BAL samples quality allowing a reliable mineralogical analysis. METHODS: All BAL samples analyzed between 2018 and 2020 in the Asbestos Fibers and Particles Laboratory from Paris City were included. They were read by an experienced cyto-pathologist and validated according to their representativeness of the alveolar region compartment. Univariate and stratified analyses were conducted to identify factors associated with the samples' cytological quality. RESULTS: On the 780 samples included, 64.4% were deemed of good cytological quality and 17.9% were not interpretable. Injected volume and BAL yield (recovery volume on injected volume ratio) were associated with cytological quality. Injecting at least 100mL with a ≥60% yield or injecting at least 150mL with a ≥30% yield allowed having a good proportion of BAL with sufficient cytological quality. CONCLUSIONS: Injected volume greater than 100mL with sufficient BAL yield are essential factors to ensure a reliable mineralogical analysis of BAL samples.
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Amianto , Amianto/análise , Lavagem Broncoalveolar , Líquido da Lavagem Broncoalveolar , Laboratórios , Minerais/análiseRESUMO
BACKGROUND: etiological investigations are not done for all adult patients with bronchiectasis because of the availability and interpretation of tests. The aim of the study was to elaborate a score to identify patients at high risk of having cystic fibrosis or primary ciliary dyskinesia (CF/PCD), which require appropriate management. METHODS: diagnostic work-ups were carried out on a French monocenter cohort, and results were subjected to logistic-regression analyses to identify the independent factors associated with CF/PCD diagnosis and, thereby, elaborate a score to validate in a second cohort. RESULTS: among 188 patients, 158 had no obvious diagnosis and were enrolled in the algorithm-construction group. In multivariate analyses, age at symptom onset (8.69 (2.10-35.99); p = 0.003), chronic ENT symptoms or diagnosed sinusitis (10.53 (1.26-87.57); p = 0.03), digestive symptoms or situs inversus (5.10 (1.23-21.14); p = 0.025), and Pseudomonas. aeruginosa and/or Staphylococcus aureus isolated from sputum (11.13 (1.34-92.21); p = 0.02) are associated with CF or PCD. Receiver operating characteristics curve analysis, using a validation group of 167 patients with bronchiectasis, confirmed the score's performance with AUC 0.92 (95% CI: 0.84-0.98). CONCLUSIONS: a clinical score may help identify adult patients with bronchiectasis at higher risk of having CF or PCD.
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BACKGROUND: In France, data regarding epidemiology and management of severe asthma are scarce. The objective of this study was to describe asthma phenotypes using a cluster analysis in severe asthmatics recruited in a real world setting. METHODS: The study design was prospective, observational and multicentric. The patients included were adults with severe asthma (GINA 4-5) followed-up in French Non Academic Hospital between May 2016 and June 2017. One hundred and seven physicians included 1502 patients. Both sociodemographic and clinical variables were collected. Hierarchical cluster analysis was performed by the Ward method followed by k-means cluster analysis on a population of 1424 patients. RESULTS: Five clusters were identified: cluster 1 (n = 690, 47%) called early onset allergic asthma (47.5% with asthma before 12 years), cluster 2 (n = 153, 10.5%): obese asthma (63.5% with BMI > 30 kg/m2), cluster 3 (n = 299, 20.4%): late-onset asthma with severe obstructive syndrome (89% without atopy), cluster 4 (n = 143, 9.8%): eosinophilic asthma (51.7% had more than 500 eosinophils/mm3), and cluster 5 (n = 139, 9.5%): aspirin sensitivity asthma (63% had severe asthma attacks). CONCLUSIONS: In our population of adults with severe asthma followed by pulmonologists, five distinct phenotypes were identified and are quite different from those mentioned in previous studies.
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Asma/diagnóstico , Adolescente , Adulto , Idade de Início , Asma/epidemiologia , Asma/fisiopatologia , Asma Induzida por Aspirina/diagnóstico , Asma Induzida por Aspirina/epidemiologia , Índice de Massa Corporal , Criança , Análise por Conglomerados , Comorbidade , Eosinofilia/diagnóstico , Eosinofilia/epidemiologia , Feminino , Volume Expiratório Forçado , França/epidemiologia , Humanos , Pulmão/fisiopatologia , Masculino , Pessoa de Meia-Idade , Obesidade/diagnóstico , Obesidade/epidemiologia , Fenótipo , Estudos Prospectivos , Fatores de Risco , Índice de Gravidade de Doença , Adulto JovemRESUMO
INTRODUCTION: Idiopathic pulmonary fibrosis (IPF) is the most common and severe interstitial lung disease (ILD). It is a progressive disease that requires a regular follow-up: clinical examination, pulmonary function testing (PFT) and CT scan, which is performed yearly in France. These exams have two major disadvantages: patients with severe dyspnoea have difficulties to perform PFT and repeated CT scans expose to high dose of radiations. Considering these limits, it would be relevant to develop new tools to monitor the progression of IPF lesions. Three main signs have been described in ILD with lung ultrasound (LUS): the number of B lines, the irregularity and the thickening of the pleural line. Cross-sectional studies already correlated the intensity of these signs with the severity of fibrosis lesions on CT scan in patients with IPF, but no prospective study described the evolution of the three main LUS signs, nor the correlation between clinical evaluation, PFT and CT scan. Our hypothesis is that LUS is a relevant tool to highlight the evolution of pulmonary lesions in IPF. The main objective of our study is to show an increase in one or more of the three main LUS signs (total number of B lines, pleural line irregularity score and pleural line thickness) during the follow-up. METHODS: ThOracic Ultrasound in Idiopathic Pulmonary Fibrosis Evolution is a French prospective, multicentric and non-interventional study. Every 3 months, patients with IPF will have a clinical examination, PFT and LUS. CT data will be collected if the CT scan is performed within 3 months before the inclusion; the second CT scan will be performed from 9 to 12 months after the inclusion. The presence, location and severity of LUS signs will be recorded for each patient, and their correlation with clinical, functional and CT scan evolution will be evaluated. 30 patients will be enrolled. ETHICS AND DISSEMINATION: The protocol was approved by the French Research Ethics Committee (Comité de Protection des Personnes SUD OUEST ET OUTRE MER II, reference RIPH3-RNI19-TOUPIE) on 11 April 2019. Results will be disseminated via peer-reviewed publication and presentation at international conferences. TRIAL REGISTRATION NUMBER: NCT03944928;Pre-results.
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Fibrose Pulmonar Idiopática , Estudos Transversais , França , Humanos , Fibrose Pulmonar Idiopática/diagnóstico por imagem , Estudos Prospectivos , UltrassonografiaRESUMO
BACKGROUND: The decision-making on antiplatelet drug withdrawal or continuation before performing a pleural procedure is based on the balance between the risk of bleeding associated with the antiplatelet therapy and the risk of arterial thrombosis due to its interruption. Knowledge on antiplatelet therapy-associated risk of bleeding after pleural procedures is lacking. RESEARCH QUESTION: Is the risk of bleeding associated with antiplatelet drugs increased in patients undergoing pleural procedures? STUDY DESIGN AND METHODS: We conducted a French multicenter cohort study in 19 centers. The main outcome was the occurrence of bleeding, defined as hematoma, hemoptysis, or hemothorax, during the 24 h following a pleural procedure. Serious bleeding events were defined as bleeding requiring blood transfusion, respiratory support, endotracheal intubation, embolization, or surgery, or as death. RESULTS: A total of 1,124 patients was included (men, 66%; median age, 62.6 ± 27.7 years), of whom 182 were receiving antiplatelet therapy and 942 were not. Fifteen patients experienced a bleeding event, including eight serious bleeding events. The 24-h incidence of bleeding was 3.23% (95% CI, 1.08%-5.91%) in the antiplatelet group and 0.96% (95% CI, 0.43%-1.60%) in the control group. The occurrence of bleeding events was significantly associated with antiplatelet therapy in univariate analysis (OR, 3.44; 95% CI, 1.14-9.66; P = .021) and multivariate analysis (OR, 4.13; 95% CI, 1.01-17.03; P = .044) after adjusting for demographic data and the main risk factors for bleeding. Likewise, antiplatelet therapy was significantly associated with serious bleeding in univariate analysis (OR, 8.61; 95% CI, 2.09-42.3; P = .003) and multivariate analysis (OR, 7.27; 95% CI, 1.18-56.1; P = .032) after adjusting for the number of risk factors for bleeding. INTERPRETATION: Antiplatelet therapy was associated with an increased risk of post-pleural procedure bleeding and serious bleeding. Future guidelines should take into account these results for patient safety.
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Hemorragia/induzido quimicamente , Inibidores da Agregação Plaquetária/uso terapêutico , Doenças Pleurais/terapia , Adulto , Idoso , Biópsia , Tubos Torácicos , Estudos de Coortes , Feminino , França , Humanos , Masculino , Pessoa de Meia-Idade , Risco , ToracenteseRESUMO
BACKGROUND: Real-world data describing management of patients with severe asthma are limited. To address this issue, we conducted FASE-CPHG (France Asthme Sevère - Collège des Pneumologues des Hôpitaux Généraux), a descriptive, multicentric, and observational cross-sectional study. METHODS: French pulmonologists from nonacademic hospitals completed questionnaires on patient characteristics and ongoing asthma treatment for severe asthmatic patients observed during the inclusion period. In addition, we collected data from patients via self-assessment questionnaires. FINDINGS: 104 physicians recruited 1502 patients within 1â year. The mean age of the 1465 patients analysed was 54.4±16.1â years. Severe asthmatic patients were more frequently female (63%), with a history of atopy (65%). Most patients remained poorly controlled or uncontrolled, with an important difference between physicians' opinion and the Global Initiative for Asthma criteria (63% versus 96%). The most common comorbidities included ear, nose and throat diseases (59% of cases); anxiety (40%); and gastro-oesophageal reflux disease (39%). Allergic sensitisation tests and/or blood eosinophil count evaluation, and spirometry were performed in 92% and 98% of patients, respectively. The mean eosinophil count and total serum IgE were 437â cells·mm-3 and 546â UI·L-1, respectively. In addition to high doses of inhaled corticosteroids plus long-acting ß2-agonists, patients were receiving leukotriene receptor antagonists (52%), anticholinergic drugs (34%), anti IgE (27%) and oral corticosteroids (17%); 65% adhered to their treatment. INTERPRETATION: This study provides insight into the characteristics and management of severe asthma in France and may help improve knowledge on this pathology, which represents a high burden to healthcare.
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Idiopathic nephrotic syndrome comprises several podocyte diseases of unknown origin that affect the glomerular podocyte, which controls the permeability of the filtration barrier in the kidney to proteins. It is characterized by the daily loss of more than 3 g of protein in urine and the lack of inflammatory lesions or cell infiltration. We found that the abundance of c-mip (c-maf inducing protein) was increased in the podocytes of patients with various acquired idiopathic nephrotic syndromes in which the podocyte is the main target of injury. Mice engineered to have excessive c-mip in podocytes developed proteinuria without morphological alterations, inflammatory lesions, or cell infiltration. Excessive c-mip blocked podocyte signaling by preventing the interaction of the slit diaphragm transmembrane protein nephrin with the tyrosine kinase Fyn, thereby decreasing phosphorylation of nephrin in vitro and in vivo. Moreover, c-mip inhibited interactions between Fyn and the cytoskeletal regulator N-WASP (neural Wiskott-Aldrich syndrome protein) and between the adaptor protein Nck and nephrin, potentially accounting for cytoskeletal disorganization and the effacement of foot processes seen in idiopathic nephrotic syndromes. The intravenous injection of small interfering RNA targeting c-mip prevented lipopolysaccharide-induced proteinuria in mice. Together, these results identify c-mip as a key component in the molecular pathogenesis of acquired podocyte diseases.
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Proteínas de Transporte/fisiologia , Podócitos/fisiologia , Proteinúria/fisiopatologia , Transdução de Sinais/fisiologia , Proteínas Adaptadoras de Transdução de Sinal , Animais , Proteínas de Transporte/biossíntese , Proteínas de Transporte/genética , Humanos , Proteínas de Membrana/metabolismo , Camundongos , Camundongos Transgênicos , Fosforilação , Podócitos/metabolismo , Ligação Proteica , Proteínas Proto-Oncogênicas c-fyn/metabolismo , Interferência de RNA , Proteína Neuronal da Síndrome de Wiskott-Aldrich/metabolismoRESUMO
BACKGROUND: There are limited series concerning Dieulafoy disease of the bronchus. We describe the clinical presentation of a series of 7 patients diagnosed with Dieulafoy disease of the bronchus and provide information about the pathological diagnosis approach. PATIENTS AND METHODS: A retrospective review of patients who underwent surgery for massive and unexplained recurrent hemoptysis in a referral center during a 11-year period. RESULTS: Seven heavy smoker (49 pack years) patients (5 males) mean aged 54 years experienced a massive hemoptysis (350-1000 ml) unrelated to a known lung disease and frequently recurrent. Bronchial contrast extravasation was observed in 3 patients, combining both CT scan and bronchial arteriography. Efficacy of bronchial artery embolization was achieved in 40% of cases before surgery. Pathological examination demonstrated a minute defect in 3 cases and a large and dysplasic superficial bronchial artery in the submucosa in all cases. CONCLUSION: Dieulafoy disease should be suspected in patients with massive and unexplained episodes of recurrent hemoptysis, in order to avoid hazardous endoscopic biopsies and to alert the pathologist if surgery is performed.
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Artérias Brônquicas/patologia , Broncopatias/diagnóstico , Broncopatias/terapia , Hemoptise/diagnóstico , Hemoptise/terapia , Adulto , Idoso , Angiografia , Artérias Brônquicas/cirurgia , Broncopatias/complicações , Broncoscopia , Embolização Terapêutica , Feminino , Hemoptise/etiologia , Humanos , Pulmão/irrigação sanguínea , Pulmão/patologia , Pulmão/cirurgia , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Tomografia Computadorizada por Raios X , Resultado do Tratamento , Vasoconstritores/uso terapêuticoRESUMO
Pleurisy is a common syndrome revealing or complicating numerous diseases. It is suspected on dyspnea or chest pain, with or without dullness to percussion. Diagnosis is confirmed almost always by chest roentgenogram but CT scan and echography can help in case of small pleural effusion. Etiologic research is based on pleural fluid analysis. The dosages of protein and LDH separate transudate from exsudate. Cellular differential count and bacterial and mycobacterial culture must systematically be performed. Other pleural analysis are discussed when specific illness are suspected. In case of exsudative pleural effusion, histological examination of pleural tissue sample is often necessary. Despite the etiological search, some pleurisy remains without specific diagnosis and necessitate a prolonged follow up with sometimes repeated thoracocentesis or biopsy. Treatment of pleurisy is based on the treatment of the underlying disease and the evacuation of pleural fluid.
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Pleurisia/diagnóstico , Biópsia , Humanos , Pleura/diagnóstico por imagem , Pleura/patologia , Pleurisia/etiologia , Radiografia Torácica , Toracoscopia , UltrassonografiaRESUMO
BACKGROUND: There is no recognized medical treatment for chronic pulmonary aspergillosis (CPA) apart from surgery in patients with simple aspergilloma. To evaluate the efficacy of voriconazole in this setting, we conducted a retrospective multicenter study over a 3-year period. METHODS: For inclusion in the study, patients had to have received voriconazole for treatment of confirmed or probable CPA with a follow-up of at least 6 months. Clinical, radiologic, and mycologic data were collected at baseline, every 2 to 3 months, and at the end of treatment or at the date point. RESULTS: Twenty-four patients were included in the study, among which 9 patients presented with chronic cavitary pulmonary aspergillosis and 15 presented with chronic necrotizing pulmonary aspergillosis (CNPA). Voriconazole was given as a first-line treatment to 13 patients. The median duration of treatment and follow-up were 6.5 and 10 months, respectively. Three patients had to stop treatment with voriconazole because of toxicity. Symptoms and imagery findings were improved in 16 of 24 patients and 17 of 24 patients, respectively, at the end of follow-up. Mycology, which was positive at baseline in 21 of 23 patients, was negative in 18 of 19 patients at the end of follow-up; serologic test results were also negative in 6 of 19 evaluable patients, all of whom had CNPA. Improved radioclinical findings and mycologic eradication were observed at the end of follow-up in 11 of 19 patients (58%). Patients in whom the disease was controlled had a significantly longer median duration of treatment than patients in whom it was uncontrolled (9 vs 6 months, respectively; p = 0.04). CONCLUSION: Voriconazole provides effective treatment of CPA with an acceptable level of toxicity.
Assuntos
Antifúngicos/uso terapêutico , Aspergilose Broncopulmonar Alérgica/tratamento farmacológico , Imunocompetência , Pirimidinas/uso terapêutico , Triazóis/uso terapêutico , Adulto , Idoso , Antifúngicos/efeitos adversos , Aspergilose Broncopulmonar Alérgica/imunologia , Doença Crônica , Estudos de Coortes , Relação Dose-Resposta a Droga , Feminino , Seguimentos , Humanos , Pulmão/imunologia , Pulmão/microbiologia , Masculino , Pessoa de Meia-Idade , Pirimidinas/efeitos adversos , Estudos Retrospectivos , Triazóis/efeitos adversos , VoriconazolRESUMO
STUDY OBJECTIVES: Flexible bronchoscopy (FB) is an invasive procedure associated with patient discomfort and frequent nose pain. A simple sedation procedure that does not require the intervention of an anesthetist is of interest. The aim of this prospective, randomized, double-blind study was to assess efficiency of nitrous oxide inhalation on the reduction of FB-induced discomfort in adult patients. DESIGN AND SETTINGS: Two hundred six patients were randomized to receive either a prefixed equimolar nitrous oxide/oxygen mixture (N2O) or a prefixed equimolar nitrogen and oxygen mixture (control). The primary outcome was stress as assessed by pulse rate and systemic BP during the procedure. Secondary outcomes were self-assessed pain using a visual analog scale (VAS) and patient satisfaction based on a questionnaire. Adverse events were recorded. RESULTS: A significant increase in BP was observed only in the control group (p = 0.003), while pulse rate values did not differ between the two groups. As assessed by the VAS, pain was lower in the N2O group as compared to placebo (p = 0.02). Nose pain and cough were also significantly reduced by N2O. Adverse events, mostly anxiety, were reported in 10 patients. CONCLUSION: These results indicate that equimolar N2O inhalation is efficient in reducing patient discomfort and may be an alternative to general anesthesia.