Viral Triggers in Pediatric Lung Transplantation
Lung TransplantBronchiolitis Obliterans1 moreThe purpose of this study is to determine whether respiratory viral infections increase the risk of bronchiolitis obliterans syndrome (BOS), obliterative bronchiolitis (OB), death, or retransplantation in children who have received lung transplants.
Correlation of Lung Biopsy, BAL, and High Resolution CT Scan in Lung Transplantation
Bronchiolitis Obliterans SyndromeA major source of graft failure and dysfunction in lung transplantation is known to be bronchiolitis obliterans (BO)and its clinical correlate called bronchiolitis obliterans syndrome(BOS). In fact, BOS is the leading cause of death in lung recipients beyond one year post transplant. Currently, our ability to assess lung injury after transplant is less than ideal. The purpose of this study is to use new computerized tomography(CT) technology, specifically , 64 bit acquisition, to detect and predict the onset of lung injuries, with the hope of finding better therapies that currently exist.
Microparticles and Bronchiolitis Obliterans Syndrome
Bronchiolitis ObliteransThe main long-term complication of lung transplantation is chronic lung allograft dysfunction (CLAD). Bronchiolitis obliterans syndrome (BOS) is the most frequent presentation of CLAD. BOS leads to a progressive loss of lung allograft function, with recurrence of dyspnea and airflow limitation. In some advanced cases, patients need a lung re transplantation. The mechanisms of BOS are not completely elucidated, and there are no early markers or specific treatment available for this condition. Microparticles (MPs) are submicron plasma membrane fragments released into the vascular compartment or the pericellular space in response to cell activation, injury or apoptosis. Broncho alveolar and circulating MPs may reflect cellular insults of the lung allografts. Therefore, MPs could be viewed either as biomarkers or as effectors of the chronic inflammatory or procoagulant processes leading to bronchiolitis obliterans syndrome. The investigators plan to include 60 patients before lung transplantation at our centre in Strasbourg (France). Follow-up will be requested at the base of usual care (spirometry, blood sampling, bronchoscopy with broncho-alveolar lavage [BAL]). The investigators will measure at one month, one, two and three year post transplantation, the total concentration of MPs in plasma and BAL and characterize their phenotype. The investigators objective is to demonstrate correlation between total MPs concentration in broncho-alveolar lavage fluid (BALF) and the occurrence of bronchiolitis obliterans syndrome at three years post lung transplantation.
INSPIRE Continuation Post-Approval Study
Bronchiolitis ObliteransTo evaluate long-term BOS-free survival outcomes of the OCS™ Lung INSPIRE Trial patients.
Immune Mediated Disorders After Allogeneic Hematopoietic Cell Transplantation
Graft vs Host DiseaseCutaneous Sclerosis1 moreThe purpose of this research study is to better understand the onset and course of graft versus host disease (GVHD)and other immune-mediated disorders after stem cell transplant.
Bronchial Inflammation in Patients With Bronchiolithis Obliterans
Bronchiolitis ObliteransThe investigators plan to study the variability of lung function parameters and bronchial inflammatory markers in 20 patients with bronchiolitis obliterans at the age of 6 to 25 years compared with an age- and sex-matched control group. The investigators will perform a pulmonary function test (body plethysmography with TLCO), and measure the fraction of exhaled nitric oxide (eNO). Further a blood sample is drawn to determine the systemic inflammation. Finally induced sputum is collected and a cell count is performed, and cells and supernatants are analyzed for inflammatory markers. This investigation will be repeated after 4-6 weeks. The aim of this study is to define baseline values and the variability of possible outcome parameters for future interventional studies.
LCI and Bronchial Inflammation in Patients With BO
Bronchiolitis ObliteransThe investigators here compare lung function parameters (RV, RV/TLC and FEF75) with the results of the Lung Clearance index (LCI) . Further this study evaluates bronchial inflammatory markers in 20 patients with bronchiolitis obliterans at the age of 6 to 28 years compared with an age- and sex-matched control group. The investigators will perform a pulmonary function test (body plethysmography with DLCO) and compare the results with the LCI. Further we will measure the fraction of exhaled nitric oxide (FeNO) and draw a blood sample to determine the level of systemic inflammation. Finally induced sputum is collected and a cell count is performed, and cells and supernatants are analyzed for inflammatory markers. This study will set baseline markers for future interventional studies.
Hyperpolarized 129Xe MRI for the Assessment of BOS With Late Onset LONIPC
Bronchiolitis ObliteransThe development of bronchiolitis obliterans syndrome (BOS) and other late onset non-infectious pulmonary complications (LONIPCs) following hematopoietic stem cell transplantation (HSCT) is associated with a significantly worse prognosis, high disease burden, and excessive health resource utilization. In this proposal, the investigators plan to examine and compare different diagnostic modalities which can provide detailed physiological and anatomical characterization of LONIPCs.
Characterization of Bronchiolitis-obliterans Syndrome (BOS) Following Lung Transplantation
Lung TransplantationBronchiolitis Obliterans SyndromeChronic organ dysfunction after lung transplantation (BOS) is the most common cause of death in long-term survivors after lung transplantation and refractory to most interventions. Early markers will be established in this project study to overcome the problem of disease recognition when impairment of graft function is already taken place. Long-term longitudinal monitoring in stable recipients of innovative markers of airway inflammation and ventilation and new imaging techniques will define different entities of chronic organ dysfunction after LTx. A database and specimen service unit for further projects will be created. Hypothesis: This project will reveal new markers and imaging tools in recipients who develop BOS after lung transplantation. These tools will allow earlier diagnosis and more accurate monitoring of the disease process. Different patterns of the disease will be characterized.
Early Detection and Management of Bronchiolitis Obliterans Syndrome Following Pediatric Hematopoietic...
Bronchiolitis Obliterans SyndromeThis study aims to determine whether or not early spirometric detection and management of obstructive lung disease with combined fluticasone/azithromycin/montelukast therapy (FAM) can attenuate declining lung function, prevent the development of bronchiolitis obliterans, and improve patient outcomes following hematopoietic stem cell transplant.