Altered Epithelial-Mesenchymal Progenitor States Lead to Matrix Deposition, Tissue Inflammation, and Transitional Epithelial State in Congenital Diaphragmatic Hernia

CDH lung mesenchymal progenitors attain mesenchymal-like characteristics prematurely and there is upregulation of ECM proteins when compared to normal lung. Moreover, CDH distal epithelial cells (Krt8/18+) enter a transitional state that is seen in fibrotic lung diseases. These findings represent imbalance of EMT, and thus dysregulation of key molecular pathways, which leads to poorly developed mesenchymal and epithelial structures that we speculate causes the lung hypoplasia found in CDH.