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 Concept Videos

Chronic Pancreatitis I: Introduction01:25

Chronic Pancreatitis I: Introduction

Chronic pancreatitis is a long-standing, relapsing inflammation of the pancreas, characterized by irreversible damage to the gland. It results in progressive destruction of the pancreatic parenchyma, fibrosis, and eventual loss of both exocrine and endocrine function. The disease may evolve gradually after multiple episodes of acute pancreatitis or develop independently.EtiologyChronic pancreatitis can arise from a variety of causes:Alcohol use is the leading cause, accounting for 70–80% of...
Chronic Pancreatitis I: Introduction01:24

Chronic Pancreatitis I: Introduction

The pancreas, an elongated and flat gland situated behind the stomach, serves a vital function in digesting food and managing blood sugar levels.
Pancreatitis is the inflammation of the pancreas, which occurs when the immune system becomes active and causes swelling, pain, and disruptions in organ function. Pancreatitis can manifest as either an acute or chronic condition.
Acute pancreatitis arises suddenly and lasts for a brief duration, while chronic pancreatitis is a long-term affliction...
Chronic Pancreatitis II: Pathophysiology01:21

Chronic Pancreatitis II: Pathophysiology

Chronic pancreatitis is a progressive and irreversible inflammation of the pancreas, most often caused by long-term alcohol abuse, but it can also be related to ductal obstruction, smoking, or genetic factors.Chronic pancreatitis occurs when the pancreas is repeatedly exposed to harmful agents like alcohol, smoking, ductal obstruction, or genetic predisposition. These factors lead to the release of toxic metabolites and inflammatory cytokines, sustaining chronic inflammation in the pancreatic...
Cirrhosis II: Pathophysiology01:24

Cirrhosis II: Pathophysiology

Cirrhosis is a progressive chronic liver injury caused by prolonged inflammation, excessive fibrotic remodeling, and impaired regeneration. Over time, repeated hepatic insults disrupt the liver’s architecture and function, leading to reduced blood flow, impaired bile drainage, and diminished metabolic capacity.Pathophysiology of cirrhosisCirrhosis arises from three main responses to chronic liver damage: inflammation, immune activation, and hepatocyte death. These processes lead to structural...
Cholecystitis01:20

Cholecystitis

Cholecystitis is inflammation of the gallbladder, most commonly caused by obstruction of the cystic duct. This blockage prevents bile from draining, leading to gallbladder distension, inflammation, and potentially serious complications. This condition may present acutely or chronically and can happen with or without gallstones.EtiologyAbout 95% of cholecystitis cases are calculous, caused by gallstones blocking the cystic duct, leading to bile accumulation and inflammation of the gallbladder...
Cirrhosis I: Introduction01:23

Cirrhosis I: Introduction

Cirrhosis is a chronic, irreversible liver disease characterized by the widespread replacement of healthy liver tissue with fibrotic scar tissue and the formation of regenerative nodules.Etiology of cirrhosisCirrhosis results from sustained liver injury that triggers progressive fibrosis and structural remodeling. The underlying causes are diverse, encompassing common and less frequent clinical conditions. Regardless of the origin, all causes lead to chronic inflammation, hepatocyte loss, and...

You might also read

Related Articles

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

Sort by
Same author

Comparing the Vitreous Floaters Functional Questionnaire to the VFQ-25 in Vision Degrading Myodesopsia from Vitreous Floaters.

American journal of ophthalmology·2026
Same author

Vitreous Floaters Functional Questionnaire for Vision-Degrading Myodesopsia From Vitreous Floaters.

JAMA ophthalmology·2025
Same author

Clinical Management of Vision Degrading Myodesopsia from Vitreous Floaters: Observation vs. Limited Refractive Vitrectomy.

Ophthalmology. Retina·2025
Same author

Alterations of PINK1-PRKN signaling in mice during normal aging.

Autophagy reports·2025
Same author

Hepatic draining lymph nodes in human liver transplant: Implications in alloimmunity and tolerance.

Transplant immunology·2024
Same author

Alterations of PINK1-PRKN signaling in mice during normal aging.

bioRxiv : the preprint server for biology·2024

Related Experiment Video

Updated: Jun 6, 2026

Implementation of Non-invasive Point of Care Transient Elastography for Evaluation of Liver Disease in Pediatric Populations with Cystic Fibrosis
05:56

Implementation of Non-invasive Point of Care Transient Elastography for Evaluation of Liver Disease in Pediatric Populations with Cystic Fibrosis

Published on: August 29, 2025

Progressive familial intrahepatic cholestasis.

Tomohide Hori1, Justin H Nguyen, Shinji Uemoto

  • 1Department of Neuroscience, Mayo Clinic in Florida, Jacksonville, FL 32224, USA. hori.tomohide@mayo.edu

Hepatobiliary & Pancreatic Diseases International : HBPD INT
|December 8, 2010
PubMed
Summary

The exact causes of progressive familial intrahepatic cholestasis (PFIC) remain unknown. Further research is crucial for developing advanced therapies, particularly for PFIC type 1.

More Related Videos

A Familial Hypercholesterolemia Human Liver Chimeric Mouse Model Using Induced Pluripotent Stem Cell-derived Hepatocytes
10:56

A Familial Hypercholesterolemia Human Liver Chimeric Mouse Model Using Induced Pluripotent Stem Cell-derived Hepatocytes

Published on: September 15, 2018

Isolation of Neonatal Extrahepatic Cholangiocytes
07:54

Isolation of Neonatal Extrahepatic Cholangiocytes

Published on: June 5, 2014

Related Experiment Videos

Last Updated: Jun 6, 2026

Implementation of Non-invasive Point of Care Transient Elastography for Evaluation of Liver Disease in Pediatric Populations with Cystic Fibrosis
05:56

Implementation of Non-invasive Point of Care Transient Elastography for Evaluation of Liver Disease in Pediatric Populations with Cystic Fibrosis

Published on: August 29, 2025

A Familial Hypercholesterolemia Human Liver Chimeric Mouse Model Using Induced Pluripotent Stem Cell-derived Hepatocytes
10:56

A Familial Hypercholesterolemia Human Liver Chimeric Mouse Model Using Induced Pluripotent Stem Cell-derived Hepatocytes

Published on: September 15, 2018

Isolation of Neonatal Extrahepatic Cholangiocytes
07:54

Isolation of Neonatal Extrahepatic Cholangiocytes

Published on: June 5, 2014

Area of Science:

  • Hepatology
  • Genetics
  • Pediatric Gastroenterology

Background:

  • Three distinct types of progressive familial intrahepatic cholestasis (PFIC) have been identified.
  • The underlying etiologies for these conditions often involve unknown mechanisms.
  • Understanding these mechanisms is critical for effective patient management.

Purpose of the Study:

  • To review current knowledge on the etiologies and treatment of progressive familial intrahepatic cholestasis (PFIC).
  • To highlight the need for further research into the disease mechanisms of PFIC.
  • To emphasize the importance of developing advanced therapeutic strategies.

Main Methods:

  • A comprehensive literature search was conducted using PubMed.
  • Keywords included "progressive familial intrahepatic cholestasis" and "PFIC".
  • Relevant articles were systematically reviewed to synthesize findings.

Main Results:

  • The etiologies for all three types of PFIC remain incompletely understood.
  • Enterohepatic circulation of bile acid is a significant consideration, especially in PFIC type 1.
  • Current treatments include ursodeoxycholic acid, partial external biliary diversion, and liver transplantation, guided by disease progression.

Conclusions:

  • The unclear etiologies and disease mechanisms of PFIC necessitate urgent and detailed investigations.
  • Advanced therapeutic strategies are required to improve outcomes for PFIC patients.
  • Future research and therapeutic developments are particularly vital for PFIC type 1.