Lung Volume Reduction Coil Microbiome Study
Chronic Obstructive Pulmonary DiseaseEmphysemaStudying the microbiome of the lung in patients treated with endobronchial lung volume reduction coils for emphysema
Genetics of the Combined Pulmonary Fibrosis and Emphysema Syndrome
Combined Pulmonary Fibrosis and Emphysema SyndromePulmonary Fibrosis2 moreThe combined pulmonary fibrosis and emphysema syndrome (CPFE) individualized by our group in 2005 is characterized by an often severe dyspnea, almost exclusive male predominance, and often major, profound impairment of gas exchange contrasting with preserved lung volumes and absence of airflow obstruction, and a high risk of pre-capillary pulmonary hypertension responsible for increased mortality. Almost all patients are smokers or ex-smokers. There are some arguments in favor of genetic abnormalities in this syndrome of unknown etiology (other than smoking) including short telomeres and mutations in the telomerase complex genes. There are also emphysematous lesions, in patients with familial pulmonary fibrosis, with mutations in the SFTPC gene (surfactant protein C), and reported cases of CPFE syndrome with SFTPC mutation. No large genetic studies have been conducted to date in the CPFE syndrome. Our main hypothesis is that the proportion of subjects with short telomeres is higher among patients with CPFE syndrome than in subjects of similar age with idiopathic pulmonary fibrosis but without emphysema. It has previously been shown that mutations in the telomerase TERT or TERC genes are mostly found in people whose telomeres are abnormally short. The investigators propose to use that test to identify patients most likely carrying a mutation, and to seek, among them, the mutations in the TERT or TERC telomerase genes. The objective of the study is to compare the proportion of patients with short telomeres in the group of patients with CPFE syndrome to that of other patients (with idiopathic pulmonary fibrosis without emphysema, or with emphysema without fibrosis).
Bronchoreversibility and Radiologic Morphology of Emphysema
EmphysemaCOPDTo assess response to bronchodilation with tiotropium plus salbutamol in patients with severe emphysema and analyze relationships between bronchoreversibility response and semiquantitative computed-tomography based emphysema severity measures.
Right Ventricular Diastolic Dysfunction
COPDEmphysema1 moreHeart-lung interactions remain misunderstood whereas pulmonary and cardiac pathologies are very commonly associated. Emphysema by increasing intrathoracic pressure appears to affect cardiac function. Interestingly, previous studies have shown a link between the telediastolic volume of the right ventricle (measured by RMI) and the intensity of emphysema. Our hypothesis is that the emphysema by increasing intrathoracic pressure leads to or accentuates right cardiac diastolic dysfunction by decreasing compliance and cardiac preload. To verify this hypothesis the investigators will perform KT loop procedures in order to acquire intracardiac pressure/volume curves before and after lung volume reduction. The pressure/volume curves allow the analysis of systolic and diastolic function, cardiac contractility and loading conditions.
Evaluation of Novel Lung Function Parameters in Patients With Chronic Obstructive Pulmonary Disease...
COPDObstructive Ventilation Disorder1 moreCurrent guideline-based criteria defining COPD do not meet the challenges set by the complex pathophysiology of the disease. The investigators therefore aimed to evaluate novel or not widely used diagnostic approaches for the detection and therapeutic monitoring of COPD.
Pulmonary Hemodynamics in Patients With Severe Emphysema Pre and Post BLVR
Pulmonary HypertensionChronic Obstructive Lung DiseaseThis study is designed as a prospective study, with the primary endpoint being changes in pulmonary hemodynamic parameters after ELVR in patients with severe emphysema. Secondary endpoints will be changes in lung function parameters, exercise capacity, and QoL.
The Microbiome of Sputum, Urine and Feces in Healthy Persons and Chronic Obstructive Pulmonary Disease...
Pulmonary DiseaseChronic Obstructive2 moreExtensive studies suggest composition of microbiome of respiratory samples or lung tissues in COPD patients is different from the composition of healthy smokers. Aim of this study is to analyze composition of microbiome of various samples (e.g. feces, sputum, and urine) and to describe difference of composition between COPD patients and healthy smokers.
Management of Patients With Alpha-1 Antitrypsin Deficiency Associated Emphysema
Alpha 1-Antitrypsin DeficiencyEmphysemaThe aim of this study is to describe the natural history of patients with alpha-1 antitrypsin associated emphysema and to figure out associated prognostic factors.