Pereira TN, Walsh MJ, Lewindon PJ, Ramm GA. Paediatric cholestatic liver disease: Diagnosis, assessment of disease progression and mechanisms of fibrogenesis. World J Gastrointest Pathophysiol 2010; 1(2): 69-84 [PMID: 21607144 DOI: 10.4291/wjgp.v1.i2.69]
Corresponding Author of This Article
Grant A Ramm, PhD, Associate Professor, Head, Hepatic Fibrosis Group, the Queensland Institute of Medical Research, PO Box Royal Brisbane and Women’s Hospital, Herston, Brisbane, QLD 4029, Australia. grant.ramm@qimr.edu.au
Article-Type of This Article
Topic Highlight
Open-Access Policy of This Article
This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
World J Gastrointest Pathophysiol. Jun 15, 2010; 1(2): 69-84 Published online Jun 15, 2010. doi: 10.4291/wjgp.v1.i2.69
Paediatric cholestatic liver disease: Diagnosis, assessment of disease progression and mechanisms of fibrogenesis
Tamara N Pereira, Meagan J Walsh, Peter J Lewindon, Grant A Ramm
Tamara N Pereira, Meagan J Walsh, Peter J Lewindon, Grant A Ramm, The Hepatic Fibrosis Group, The Queensland Institute of Medical Research, Brisbane, QLD 4029, Australia Peter J Lewindon, Department of Gastroenterology, Royal Children’s Hospital, Brisbane, QLD 4029, Australia
Author contributions: Pereira TN and Walsh MJ contributed equally, researched the content and wrote the manuscript; Lewindon PJ provided clinical editing and assisted with writing the manuscript; and Ramm GA provided scientific editing and assisted with writing the manuscript.
Supported by a Project Grant from the National Health and Medical Research Council of Australia (NHMRC #496602 to GAR and PJL). Dr. Tamara Pereira is Supported by the Phillip Bushell Foundation Post-Doctoral Research Fellowship from the Gastroenterological Society of Australia. Associate Professor Grant A Ramm is Supported by a NHMRC Senior Research Fellowship (NHMRC #552409)
Correspondence to: Grant A Ramm, PhD, Associate Professor, Head, Hepatic Fibrosis Group, the Queensland Institute of Medical Research, PO Box Royal Brisbane and Women’s Hospital, Herston, Brisbane, QLD 4029, Australia. grant.ramm@qimr.edu.au
Telephone: +61-7-33620177 Fax: +61-7-33620191
Received: February 9, 2010 Revised: March 26, 2010 Accepted: April 2, 2010 Published online: June 15, 2010
Abstract
Cholestatic liver disease causes significant morbidity and mortality in children. The diagnosis and management of these diseases can be complicated by an inability to detect early stages of fibrosis and a lack of adequate interventional therapy. There is no single gold standard test that accurately reflects the presence of liver disease, or that can be used to monitor fibrosis progression, particularly in conditions such as cystic fibrosis. This has lead to controversy over how suspected liver disease in children is detected and diagnosed. This review discusses the challenges in using commonly available methods to diagnose hepatic fibrosis and monitor disease progression in children with cholestatic liver disease. In addition, the review examines the mechanisms hypothesised to be involved in the development of hepatic fibrogenesis in paediatric cholestatic liver injury which may ultimately aid in identifying new modalities to assist in both disease detection and therapeutic intervention.
