Cited by 287Liver Diseases and ImmunityLiver physiology and pathologyPediatric Hepatobiliary Diseases and Treatments

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Pathogenesis of Cholestatic Liver Disease and Therapeutic Approaches

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Hirschfield, Gideon M., Heathcote, E. Jenny, Gershwin, M. Eric·2010·Gastroenterology

DOI 10.1053/j.gastro.2010.09.004

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Abstract

Cholestatic liver disorders are caused by genetic defects, mechanical aberrations, toxins, or dysregulations in the immune system that damage the bile ducts and cause accumulation of bile and liver tissue damage. They have common clinical manifestations and pathogenic features that include the responses of cholangiocytes and hepatocytes to injury. We review the features of bile acid transport, tissue repair and regulation, apoptosis, vascular supply, immune regulation, and cholangiocytes that are associated with cholestatic liver disorders. We now have a greater understanding of the physiology of cholangiocytes at the cellular and molecular levels, as well as genetic factors, repair pathways, and autoimmunity mechanisms involved in the pathogenesis of disease. These discoveries will hopefully lead to new therapeutic approaches for patients with cholestatic liver disease.

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1 retracted

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Authors: Hirschfield, G. M., Heathcote, E. J., & Gershwin, M. E.
Affiliations: University of Toronto (CA)
University of California, Davis (US)
Corresponding author: Gershwin, M. Eric
ORCID: Hirschfield, Gideon M.
Gershwin, M. Eric
Venue: Gastroenterology
Publisher: Elsevier BV
Published: 2010-11
Volume: 139
Issue: 5
Pages: 1481-1496
ISSN: 0016-5085 (ISSN-L)
DOI: 10.1053/j.gastro.2010.09.004
OpenAlex: W2079853302
Cited by: 287
References: 187
Citations by year: 2026: 15 · 2025: 20 · 2024: 21 · 2023: 21 · 2022: 16 · 2021: 19 · 2020: 21 · 2019: 16 · 2018: 19 · 2017: 17 · 2016: 14 · 2015: 14 · 2014: 21 · 2013: 16 · 2012: 21
FWCI: 2.32
Type: journal article
Language: en
Open access: bronze
doi.org
Licence: www.elsevier.com/tdm/userlicense/1.0/ (tdm)
Locations: Gastroenterology (publishedVersion)
PubMed (publishedVersion)
University of Birmingham Research Portal (University of Birmingham) (submittedVersion)
Funders: Canadian Institutes of Health Research (MOP74621)
National Institute of Diabetes and Digestive and Kidney Diseases (DK 39588)
Topics: Health Sciences › Medicine › Hepatology › Liver Diseases and Immunity
Health Sciences › Medicine › Hepatology › Liver physiology and pathology
Health Sciences › Medicine › Surgery › Pediatric Hepatobiliary Diseases and Treatments
Keywords: cholestatic liver disease, pathogenesis, cholangiocytes, bile acid transport, cholestasis, bile duct injury, hepatocyte injury, tissue repair, apoptosis, blood supply, immune regulation, autoimmunity, genetic disorders, treatment options
MeSH terms: Animals, Bile, Cholagogues and Choleretics, Cholestasis, Humans, Immunity, Cellular, Immunosuppressive Agents, Liver Cirrhosis, Biliary, Prognosis, Risk Factors, Ursodeoxycholic Acid, Cytokines, Apoptosis, Hepatocytes

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