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Related Concept Videos

Diseases of the Liver and Gallbladder01:26

Diseases of the Liver and Gallbladder

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Liver and gallbladder diseases are a significant health concern, with prominent conditions including cirrhosis, hepatitis, non-alcoholic fatty liver disease (NAFLD), and gallstones. Jaundice is a common manifestation of liver and biliary disease.
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Cirrhosis I: Introduction01:23

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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...
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Chronic Bowel Disorders: Introduction01:17

Chronic Bowel Disorders: Introduction

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Chronic bowel diseases are a group of long-term conditions affecting the digestive tract, characterized by inflammation and damage to the gut lining. These conditions primarily include irritable bowel syndrome and inflammatory bowel disease.
Irritable Bowel Syndrome (IBS) is a common disorder affecting the gastrointestinal tract. The distinctive feature is recurrent abdominal pain associated with altered bowel movements, manifesting as constipation, diarrhea, or fluctuating between both. The...
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Cholecystitis01:20

Cholecystitis

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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...
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Chronic Pancreatitis II: Pathophysiology01:21

Chronic Pancreatitis II: Pathophysiology

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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...
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Cirrhosis II: Pathophysiology01:24

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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...
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Robotic Left Hepatectomy using Indocyanine Green Fluorescence Imaging for an Intrahepatic Complex Biliary Cyst
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Primary biliary cirrhosis in 2014.

Avegail Flores1, Marlyn J Mayo

  • 1Division of Digestive and Liver Diseases, University of Texas Southwestern, Dallas, Texas, USA.

Current Opinion in Gastroenterology
|March 28, 2014
PubMed
Summary
This summary is machine-generated.

Primary biliary cirrhosis (PBC) is an autoimmune liver disease. Recent advances in genetics and nuclear hormone receptor research offer new treatment strategies beyond ursodiol for patients with PBC.

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Area of Science:

  • Hepatology
  • Immunology
  • Genetics

Background:

  • Primary biliary cirrhosis (PBC) is a chronic cholestatic liver disease characterized by autoimmune destruction of intrahepatic bile ducts.
  • The autoimmune basis of PBC was recognized in the 1980s with the identification of antimitochondrial antibodies.
  • Ursodiol, effective in delaying progression, is not a cure for PBC.

Purpose of the Study:

  • To review the historical understanding and recent advances in Primary Biliary Cirrhosis (PBC).
  • To highlight the evolving therapeutic landscape for PBC.
  • To inform clinicians on predicting disease progression and emerging treatments.

Main Methods:

  • Review of historical literature and recent scientific advances in PBC research.
  • Analysis of genome-wide association studies (GWAS) enabled by rapid sequencing technologies.
  • Elucidation of nuclear hormone receptor roles in bile salt metabolism.

Main Results:

  • Genome-wide association studies have identified genetic factors contributing to PBC.
  • Understanding nuclear hormone receptors has led to novel therapeutic targets for cholestatic diseases.
  • New insights into bile acid metabolism are guiding the development of innovative treatments.

Conclusions:

  • Clinicians can now better identify PBC patients likely to progress despite ursodiol.
  • Emerging treatments include novel bile acid therapies and immunosuppressants.
  • Awareness of genetic factors is crucial for understanding PBC development.