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Alveolar epithelial disintegrity in pulmonary fibrosis.
| Content Provider | Semantic Scholar |
|---|---|
| Author | Kulkarni, Tejaswini Andrade, Joao A. De Zhou, Yong Luckhardt, Tracy Thannickal, Victor J. |
| Copyright Year | 2016 |
| Abstract | Idiopathic pulmonary fibrosis (IPF) is a chronic lung disease characterized by progressive decline in lung function, resulting in significant morbidity and mortality. Current concepts of the pathogenesis of IPF primarily center on dysregulated epithelial cell repair and altered epithelial-mesenchymal communication and extracellular matrix deposition following chronic exposure to cigarette smoke or environmental toxins. In recent years, increasing attention has been directed toward the role of the intercellular junctional complex in determining the specific properties of epithelia in pulmonary diseases. Additionally, recent genomewide association studies suggest that specific genetic variants predictive of epithelial cell dysfunction may confer susceptibility to the development of sporadic idiopathic pulmonary fibrosis. A number of genetic disorders linked to pulmonary fibrosis and familial interstitial pneumonias are associated with loss of epithelial integrity. However, the potential links between extrapulmonary clinical syndromes associated with defects in epithelial cells and the development of pulmonary fibrosis are not well understood. Here, we report a case of hereditary mucoepithelial dysplasia that presented with pulmonary fibrosis and emphysema on high-resolution computed tomography. This case illustrates a more generalizable concept of epithelial disintegrity in the development of fibrotic lung diseases, which is explored in greater detail in this review article. |
| Starting Page | 221A |
| Ending Page | 221A |
| Page Count | 1 |
| File Format | PDF HTM / HTML |
| Alternate Webpage(s) | http://ajplung.physiology.org/content/ajplung/early/2016/05/24/ajplung.00115.2016.full.pdf |
| PubMed reference number | 27233996v1 |
| Alternate Webpage(s) | https://doi.org/10.1152/ajplung.00115.2016 |
| DOI | 10.1152/ajplung.00115.2016 |
| Journal | American journal of physiology. Lung cellular and molecular physiology |
| Volume Number | 311 |
| Issue Number | 2 |
| Language | English |
| Access Restriction | Open |
| Subject Keyword | Chronic lung disease Extracellular Matrix Hereditary Diseases Idiopathic Pulmonary Fibrosis Lung diseases Morbidity - disease rate Pneumonia, Interstitial Pneumonitis Pulmonary Alveolar Proteinosis Pulmonary Emphysema Respiratory physiology Toxin Urban Schosser Spohn syndrome X-Ray Computed Tomography |
| Content Type | Text |
| Resource Type | Article |
