Each of the projects in this SCOR grant application seeks to assess relationships between the in vivo and in vitro models of lung morphogenesis and repair and the pathogenesis of lung disease in neonates and adults. Our SCOR investigators have collaborated actively in numerous clinical studies during the past five years and now seek to establish a clinical core for tissue collection, storage of clinical data, management and for scientific interactions focused to correlations between basic science findings developed with SCOR projects and clinical disease. The Core will obtain, catalog and store biopsy and autopsy tissue and clinical data from infants with neonatal lung diseases including congenital malformations cystadenomatoid malformation, congenital alveolar hypoplasia/acinar hypoplasia, diaphragmatic hernia, surfactant protein B deficiency, desquamating interstitial pneumonitis, neonatal alveolar proteinosis and bronchopulmonary dysplasia. Adult biopsy tissues obtained from patients with ARDS, IPF sarcoidosis and pulmonary alveolar proteinosis will be stored and maintained for correlation with the molecular markers developed within the SCOR projects. These tissues will be used to assess the potential roles of growth factor, cytokine systems, and transcription factors studied in the individual projects in the developing, injured and recovering lung. Tissue samples will be prepared for immunochemistry, in situ hybridization and morphology. DNA samples will be obtained and stored for genetic analysis of potential target gene abnormalities that may be associated with the clinical disease. Clinical samples and data will be used to test the hypothesis that the factors and mechanisms controlling lung growth and differentiation are also recapitulated following lung injury and during normal and abnormal repair processes.
