TRANSPIRE: Lung Injury in a Longitudinal Cohort of Pediatric HSCT Patients
Hematopoietic Stem Cell Transplant (HSCT)Diffuse Alveolar Hemorrhage3 moreHematopoietic stem cell transplant (HSCT) is an effective but toxic therapy and pulmonary morbidity affects as many as 25% of children receiving transplant. Early pulmonary injury includes diffuse alveolar hemorrhage (DAH), thrombotic microangiopathy (TMA) interstitial pneumonitis (IPS) and infection, while later, bronchiolitis obliterans is a complication of chronic GVHD associated with severe morbidity and mortality. Improved diagnosis and treatment of pulmonary complications are urgently needed as survival after HSCT improves, and as HSCT is increasingly used for non-malignant disorders such as sickle cell disease. Currently, there are large and important gaps in the investigator's knowledge regarding incidence, etiology and optimal treatment of pulmonary complications. Moreover, young children unable to perform spirometry are often diagnosed late, and strategies for monitoring therapeutic response are limited. This is a prospective multi-institutional cohort study in pediatric patients undergoing allogeneic (alloHSCT) or autologous hematopoietic stem cell transplantation (autoHSCT). Assembly of a large prospective uniformly screened cohort of children receiving HSCT, together with collection of biological samples, will be an effective strategy to identify mechanisms of lung injury, test novel diagnostic strategies for earlier diagnosis, and novel treatments to reduce morbidity and mortality from lung injury after transplant.
Evaluating Clonogenic Epithelial Cell Populations in Patients With Bronchiolitis Obliterans Syndrome...
Bronchiolitis ObliteransThis study investigates a type of cell, called abnormal clonogenic epithelial cells, in patients with bronchiolitis obliterans syndrome who have had an donor stem cell transplant or a lung transplant. Learning more about clonogenic cells in these patients may help doctors to detect signs of bronchiolitis obliterans syndrome earlier in future patients.
Prospective Evaluations of Infectious Complication in Lung Transplant Recipients
Disorder Related to Lung TransplantationBronchiolitis ObliteransThe primary aim of this study is to determine prospectively the viral and C. pneumoniae infection prevalence and outcomes of infections in lung transplant recipients. The study will also determine the correlation of C. pneumoniae infection with the development of obliterans in lung transplant recipients.
The Longitudinal Impact of Respiratory Viruses on Bronchiolitis Obliterans Syndrome After Allogeneic...
Bronchiolitis Obliterans SyndromeHematopoietic and Lymphoid Cell NeoplasmThis observational trial studies whether respiratory viruses are the cause of lung disease (bronchiolitis obliterans syndrome [BOS] or graft-versus-host disease of the lung) and changes in lung function in patients who have received a donor stem cell transplant. Patients with chronic graft-versus-host disease (cGVHD) are at higher risk of developing BOS. Studies have also shown that patients who had a respiratory viral illness early after their transplant are at higher risk of developing lung problems later on. Patients who are at risk and who already have BOS might benefit from being monitored more closely. Spirometry is a way of assessing a patient's lung function and is often used to diagnose lung disease. Spirometry measured at home with a simple handheld device may reduce the burden of performing pulmonary function testing at a facility and potentially help patients get their lung disease diagnosed and treated sooner.
Novel Pulmonary Function Measures for Diagnosis of Bronchiolitis Obliterans Syndrome Following Hematopoietic...
Bronchiolitis Obliterans SyndromeIn this study, we will study 129Xe-MRI and LCI as tools for diagnosis of BOS in pediatric patients who have received Hematopoietic Stem Cell Transplantation (HSCT) and have been identified as eligible for this study. Participants will be required to have vital signs collected, complete breathing tests and complete an MRI. The MRI will require participants to perform breath holds in the MRI scanner with xenon gas while being coached by a research assistant.
Measures of Respiratory Health (MRH)
Cystic FibrosisAsthma2 moreThe Lung Clearance Index, measured by multiple breath washout, is a measure of lung function that is considered a research tool in Canada as the device used to measure it is not approved by Health Canada. The study will assess lung function in patients undergoing routine lung function testing for clinical indications (Cystic Fibrosis and Other Respiratory Diseases). In addition, healthy controls of different ages will be asked to perform this lung function test to gain reference data that can be used to interpret LCI in patients with lung disease.
BElumosudil for Bronchiolitis Obliterans Prevention/Therapy (BEBOP)
Bronchiolitis Obliterans SyndromeBronchiolitis Obliterans2 moreThe goal of this research study is to test the efficacy of a novel immunosuppressive agent, belumosudil, in allogeneic hematopoietic stem cell transplant (HSCT) recipients who have been newly diagnosed or have developing (early stage) bronchiolitis obliterans syndrome (BOS). The name of the study drugs involved in this study are: Belumosudil (an immunotherapy) Fluticasone (an intranasal corticosteroid) Azithromycin (an antibiotic) Montelukast (a leukotriene receptor antagonist) Prednisone (a corticosteroid)
Efficacy Study of Azithromycin-based Therapy for Bronchiolitis Obliterans
Graft vs Host DiseaseBronchiolitis Obliterans[Study Objectives] To evaluate the efficacy of azithromycin, N-acetylcystein, and inhaled corticosteroid combination therapy in patients with bronchiolitis obliterans as a complication of allogeneic hematopoietic cell transplantation in terms of response rate at 6 months after treatment initiation based on the improvement of FEV1.
Extracorporeal Photopheresis in Patients With Bronchiolitis Obliterans Syndrome (BOS) After Lung...
Bronchiolitis ObliteransInvestigation of photopheresis as new therapy regimen in patients with bronchiolitis obliterans syndrome after lung transplantation in a controlled, randomized study
A Safety Study of Liposomal Cyclosporine A to Treat Bronchiolitis After Hematopoietic Transplant...
Bronchiolitis Obliterans Syndrome (BOS)GVHD2 morePrimary Objective: The primary objective of this study is to assess the tolerability and safety of two dose levels of aerosolized L-CsA vs placebo in addition to SoC therapy for BOS in adult allo-HSCT recipients. Secondary Objectives: The secondary objectives of this study are to assess PK and exploratory efficacy and quality of life of two dose levels of aerosolized L-CsA vs placebo in addition to SoC therapy for BOS in adult allo-HSCT recipients.