The lung defect associated with congenital diaphragmic hernia (CDH) is the main cause of mortality and morbidity in patients with CDH. However, since the lung tissue in CDH infants is not accessible, mechanisms underlying the lung defect in CDH remain an unresolved issue. This project proposes to elucidate such mechanisms using mesenchymal stromal cells (MSCs) and epithelial basal stem cells (BSCs) from patient-specific tracheal aspirate (TA) samples. Our goal is to elucidate pathogenic changes in patient-specific cell models to study disease pathophysiology and to identify novel therapeutics.
