Open AccessPaediatric cholestatic liver disease: Diagnosis, assessment of disease progression and mechanisms of fibrogenesis
Author(s) -
Tamara N. Pereira,
Meagan Walsh,
Peter Lewindon,
Grant A. Ramm
Publication year - 2010
Publication title -
world journal of gastrointestinal pathophysiology
Language(s) - English
Resource type - Journals
ISSN - 2150-5330
DOI - 10.4291/wjgp.v1.i2.69
Subject(s) - medicine , disease , fibrosis , liver disease , chronic liver disease , gold standard (test) , cystic fibrosis , intensive care medicine , hepatic fibrosis , gastroenterology , pathology , cirrhosis
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.