Serum markers of hepatic fibrogenesis in cystic fibrosis liver disease

Pereira, Tamara N., Lewindon, Peter J., Smith, Jeffery L., Murphy, Therese L., Lincoln, Douglas J., Shepherd, Ross W. and Ramm, Grant A. (2004) Serum markers of hepatic fibrogenesis in cystic fibrosis liver disease. Journal of Hepatology, 41 4: 576-583. doi:10.1016/j.jhep.2004.06.032

Author Pereira, Tamara N.
Lewindon, Peter J.
Smith, Jeffery L.
Murphy, Therese L.
Lincoln, Douglas J.
Shepherd, Ross W.
Ramm, Grant A.
Title Serum markers of hepatic fibrogenesis in cystic fibrosis liver disease
Journal name Journal of Hepatology   Check publisher's open access policy
ISSN 0168-8278
Publication date 2004
Sub-type Article (original research)
DOI 10.1016/j.jhep.2004.06.032
Volume 41
Issue 4
Start page 576
End page 583
Total pages 8
Editor M. Colombo
Place of publication Amsterdam, The Netherlands
Publisher Elsevier BV
Collection year 2004
Language eng
Subject C1
270802 Diagnostic Applications
730107 Inherited diseases (incl. gene therapy)
1103 Clinical Sciences
Formatted abstract
Hepatic fibrosis contributes to adverse outcome in cystic fibrosis (CF). Early detection of CF liver disease (CFLD) may identify patients at risk of significant complications. To evaluate the utility of serum markers to detect hepatic fibrosis in children with CFLD vs. CF patients without liver disease (CFnoLD) and controls.

Sera from 36 CFLD, 30 CFnoLD and 39 controls were assessed for tissue inhibitor of matrix metalloproteinase (NIMP) (TIMP)-1, coliagen (CL)-IV, MMP-2, hyaluronic acid (HA) and prolyl hydroxylase (PH) by enzyme immunoassay and were correlated with hepatic fibrosis score in CFLD.

TIMP-1, PH and CL-IV were increased in CFLD vs. CFnoLD and controls. Fibrosis score was negatively correlated with TIMP-1 (r=-0.34, P=0.06) and PH (r=-0.48, P=0.008). Receiver-operating characteristics analysis showed CL-IV (AUC 0.785, P<0.0001) and TIMP-1 (AUC 0.765, P<0.0001) differentiated CFLD from CFnoLD and controls, while PH (AUC 0.814, P<0.000 1) predicted early fibrogenesis. Diagnostic accuracy improved using logistic regression combining (i) CL-IV, TIMP-1, PH to identify CFLD (AUC 0.831,P<0.0001) and (ii) TIMP-1, PH to identify CFLD patients with no fibrosis (AUC 0.852, P<0.02).

Elevated TIMP-1, CL-IV, PH may be indicators of hepatic fibrogenesis in CF. Increased TIMP-1, PH may be early markers of CFLD. (C) 2004 European Association for the Study of the Liver. Published by Elsevier B.V. All rights reserved.
Keyword Collagen type IV
Cystic fibrosis liver disease
Serum markers of hepatic fibrosis
Prolyl hydroxylase
Tissue inhibitor
Q-Index Code C1

Document type: Journal Article
Sub-type: Article (original research)
Collections: Excellence in Research Australia (ERA) - Collection
2005 Higher Education Research Data Collection
School of Medicine Publications
Version Filter Type
Citation counts: TR Web of Science Citation Count  Cited 31 times in Thomson Reuters Web of Science Article | Citations
Scopus Citation Count Cited 34 times in Scopus Article | Citations
Google Scholar Search Google Scholar
Created: Wed, 15 Aug 2007, 04:03:44 EST