Jove
Visualize
Contact Us
JoVE
x logofacebook logolinkedin logoyoutube logo
ABOUT JoVE
OverviewLeadershipBlogJoVE Help Center
AUTHORS
Publishing ProcessEditorial BoardScope & PoliciesPeer ReviewFAQSubmit
LIBRARIANS
TestimonialsSubscriptionsAccessResourcesLibrary Advisory BoardFAQ
RESEARCH
JoVE JournalMethods CollectionsJoVE Encyclopedia of ExperimentsArchive
EDUCATION
JoVE CoreJoVE BusinessJoVE Science EducationJoVE Lab ManualFaculty Resource CenterFaculty Site
Terms & Conditions of Use
Privacy Policy
Policies

Related Experiment Videos

Chronic cholestatic diseases.

R Poupon1, O Chazouillères, R E Poupon

  • 1Service d'hépatogastroentérologie, Hôpital Saint-Antoine, Paris, France.

Journal of Hepatology
|March 23, 2000
PubMed
Summary
This summary is machine-generated.

Related Concept Videos

You might also read

Related Articles

Articles linked to this work by shared authors, journal, and citation graph.

Sort by
Same author

COVID-19 and chronic liver disease: results from the 1219 patients French registry.

Scientific reports·2025
Same author

Endoscopic features of low-phospholipid-associated cholelithiasis syndrome: A retrospective cohort study.

Clinics and research in hepatology and gastroenterology·2024
Same author

HLA haplotypes in primary sclerosing cholangitis patients of admixed and non-European ancestry.

HLA·2017
Same author

Liver test abnormalities in patients admitted for severe psoriasis: prevalence and associated risk factors.

Journal of the European Academy of Dermatology and Venereology : JEADV·2016
Same author

Association between IL28B polymorphism, TNFα and biomarkers of insulin resistance in chronic hepatitis C-related insulin resistance.

Journal of viral hepatitis·2015
Same author

Therapeutic approaches for hepatobiliary disorders with ursodeoxycholic acid and bile-acid derivatives.

Clinics and research in hepatology and gastroenterology·2012
Same journal

Moving Too Fast? A Meta-Analysis Calls for Caution on TNF Inhibitors as First-Line Therapy for Autoimmune Hepatitis.

Journal of hepatology·2026
Same journal

Anserine restores antibacterial immunity in cirrhosis via a protective CCL5-mediated hepatocyte-CD44<sup>+</sup>pDC crosstalk.

Journal of hepatology·2026
Same journal

Anti-CCL2-conjugated platelets attenuate early allograft and ischemia-reperfusion injury by inhibiting monocyte infiltration.

Journal of hepatology·2026
Same journal

A precision randomized trial of hepatitis C treatment support among people who inject drugs in India: The STOP-C Trial.

Journal of hepatology·2026
Same journal

Response to "Single-nucleus and spatial transcriptomic analyses reveal intra-tissue heterogeneity of PNPLA3".

Journal of hepatology·2026
Same journal

One-stop liquid biopsy: Can a single blood sample reveal the full spectrum of liver disease?

Journal of hepatology·2026
See all related articles

This review discusses chronic cholestatic diseases, focusing on diagnosis and management of primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC). It highlights advances in understanding genetic defects and treatment strategies for these liver conditions.

Area of Science:

  • Hepatology and Gastroenterology
  • Internal Medicine
  • Genetics and Molecular Biology

Background:

  • Chronic cholestatic diseases involve impaired bile acid transport due to biliary epithelium damage.
  • Primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) are major adult cholestatic disorders.
  • Advances in understanding bile secretion have identified genetic defects in progressive familial intrahepatic cholestasis (PFIC).

Purpose of the Study:

  • To provide diagnostic and management approaches for main specific cholestatic disorders.
  • To discuss recent developments in the field of cholestatic liver diseases.
  • To explore the role of genetic defects in PFIC and potential links to adult cholestasis.

Main Methods:

  • Review of diagnostic and management strategies for chronic cholestatic diseases.

Related Experiment Videos

  • Discussion of recent scientific literature on bile secretion and genetic defects.
  • Analysis of clinical presentations, survival data, and treatment responses.

    • Main Results:

    • Identification of genetic defects in PFIC, with potential implications for adult cholestasis.
    • Description of variant forms of PBC, including autoimmune cholangitis and PBC-AIH overlap syndrome.
    • Variant PSC forms include PSC-AIH overlap and small-duct PSC; cholangiocarcinoma is a complication of PSC.

    Conclusions:

    • Ursodeoxycholic acid (UDCA) improves survival in PBC; its mechanisms include anti-apoptotic effects.
    • UDCA combined with corticosteroids is effective for PBC-AIH overlap syndrome.
    • Effective medical therapy for PSC is lacking; liver transplantation is the main treatment for advanced disease, despite recurrence risk.