Pediatric lung disease: from proteinases to pulmonary fibrosis |
| |
Authors: | Chua Felix Sly Peter D Laurent Geoffrey J |
| |
Affiliation: | Centre for Respiratory Research, Royal Free and University College London Medical School, Rayne Institute, London, UK. f.chua@ucl.ac.uk |
| |
Abstract: | One distinctive outcome of interstitial lung diseases in childhood is the abnormal accumulation of pulmonary extracellular matrix. The clinical consequence of such excessive connective tissue accumulation is known as pulmonary fibrosis. While numerous aspects of its pathogenesis have become familiar, many key events involved in its inception and progression still remain unclear. There is now compelling evidence that lung damage due to uncontrolled proteolysis may help drive critical processes that regulate fibrotic matrix remodeling. In this regard, a number of proteinases have been implicated in promoting both the initial lung injury and the fibroproliferative repair that follows. This review summarizes the knowledge of how different matrix-targeting enzymes may act to influence the development of pediatric pulmonary fibrosis. Understanding the scientific basis of this complex process may highlight opportunities to limit unwanted proteolysis and the intensity of its fibrotic sequelae. |
| |
Keywords: | pediatric interstitial lung disease pulmonary fibrosis proteinases |
本文献已被 PubMed 等数据库收录! |