Pediatric lung disease: From proteinases to pulmonary fibrosis

Chua, Felix, Sly, Peter D. and Laurent, Geoffrey J. (2005) Pediatric lung disease: From proteinases to pulmonary fibrosis. Pediatric Pulmonology, 39 5: 392-401. doi:10.1002/ppul.20171

Author Chua, Felix
Sly, Peter D.
Laurent, Geoffrey J.
Title Pediatric lung disease: From proteinases to pulmonary fibrosis
Journal name Pediatric Pulmonology   Check publisher's open access policy
ISSN 8755-6863
Publication date 2005-05
Sub-type Article (original research)
DOI 10.1002/ppul.20171
Volume 39
Issue 5
Start page 392
End page 401
Total pages 10
Place of publication Hoboken, NJ, United States
Publisher John Wiley & Sons
Language eng
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 f ibrotic 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.
Keyword pediatric
interstitial lung disease
pulmonary fibrosis
Q-Index Code C1
Q-Index Status Provisional Code
Institutional Status Non-UQ

Document type: Journal Article
Sub-type: Article (original research)
Collection: Faculty of Health and Behavioural Sciences -- Publications
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Citation counts: TR Web of Science Citation Count  Cited 7 times in Thomson Reuters Web of Science Article | Citations
Scopus Citation Count Cited 10 times in Scopus Article | Citations
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Created: Wed, 17 Nov 2010, 11:22:02 EST