Role of serum markers in detection and monitoring progression of CF liver disease

Ramm, Grant A., Pereira, Tamara N. and Lewindon, Peter J. (2005). Role of serum markers in detection and monitoring progression of CF liver disease. In: Symposium Summaries of the 19th Annual North American Cystic Fibrosis Conference. The 19th Annual North American Cystic Fibrosis Conference, Baltimore, Maryland, U.S.A., (169-171). 20-23 October 2005. doi:10.1002/ppul.20314


Author Ramm, Grant A.
Pereira, Tamara N.
Lewindon, Peter J.
Title of paper Role of serum markers in detection and monitoring progression of CF liver disease
Conference name The 19th Annual North American Cystic Fibrosis Conference
Conference location Baltimore, Maryland, U.S.A.
Conference dates 20-23 October 2005
Proceedings title Symposium Summaries of the 19th Annual North American Cystic Fibrosis Conference   Check publisher's open access policy
Journal name Pediatric Pulmonology   Check publisher's open access policy
Place of Publication New York, U.S.A.
Publisher Wiley
Publication Year 2005
Sub-type Fully published paper
DOI 10.1002/ppul.20314
ISSN 1099-0496
8755-6863
Volume 40
Issue S28
Start page 169
End page 171
Total pages 3
Language eng
Formatted Abstract/Summary
First paragraph:
Liver disease is a major complication for patients with cystic fibrosis (CF), where the principal cause of morbidity and mortality is hepatic fibrosis and cirrhosis. Significant morbidity from CF-associated liver disease (CFLD) occurs in up to 17% of children with CF and cirrhosis is detected in up to 10%. Clinically significant fibrosis during life is difficult to determine as no adequate diagnostic test is available and thus diagnosis is frequently not established until serious complications such as portal hypertension are already established. Liver biopsy has for many years been the standard method for diagnosing and quantifying fibrosis, however, it can potentially be confounded by sampling error due to the focal periportal fibrosis and focal biliary cirrhosis which are characteristic of CFLD. While the precise pathobiological mechanisms responsible for liver disease in CF remain to be determined, the activated hepatic stellate cell (HSC) has been demonstrated as the cellular source of increased procollagen α1(I)mRNA expression in CFLD and thus responsible for fibrillar collagen deposition and ensuing hepatic fibrosis and cirrhosis seen in CF. The deposition of excess extracellular matrix including fibrillar collagens in hepatic fibrosis is due to an imbalance between the synthesis and degradation of these matrix components (ie., matrix re-modelling) by HSCs. Measuring the products of HSC fibrogenic activity is one of the more recent innovative approaches for monitoring fibrosis progression in chronic liver disease.


Subjects 1114 Paediatrics and Reproductive Medicine
Keyword CF liver disease
Serum markers of hepatic fibrosis
HSC fibrogenic activity
Cystic fibrosis
Fibrosis progression
Q-Index Code EX
Q-Index Status Provisional Code
Institutional Status Unknown
Additional Notes DOI is for summaries published on pages 87-189; Published in 'Symposium Summaries' section of this issue, Article no. S17.2

 
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Created: Mon, 15 Mar 2010, 11:10:12 EST by Mary-Anne Marrington on behalf of Faculty Of Health Sciences