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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

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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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Related Experiment Video

Updated: Jul 19, 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.

Rashid Mirza1, Zaigham Abbas, Nasir Hassan Luck

  • 1Department of Hepatogastroenterology, Sindh Institute of Urology and Transplantation, Karachi. rmirzamd@yahoo.com

Journal of the College of Physicians and Surgeons--Pakistan : JCPSP
|September 30, 2006
PubMed
Summary

Progressive familial intrahepatic cholestasis (PFIC) presents a significant cause of pediatric cholestatic liver disease. Early diagnosis is crucial as advanced fibrosis can obscure specific PFIC subtypes on liver biopsy.

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

Published on: June 5, 2014

Area of Science:

  • Pediatric Hepatology
  • Genetic Liver Diseases
  • Biliary System Disorders

Background:

  • Progressive familial intrahepatic cholestasis (PFIC) is a group of rare genetic disorders.
  • It leads to severe cholestatic liver disease and biliary cirrhosis in children.
  • PFIC significantly impacts pediatric liver health and requires accurate diagnosis.

Observation:

  • Three pediatric cases of PFIC are presented.
  • Diagnosis was based on family history, pruritus, cirrhosis, bile duct paucity, and an intact extrahepatic biliary tree.
  • Patients were initially misdiagnosed with extra-hepatic biliary atresia or neonatal hepatitis.

Findings:

  • Histological differentiation between PFIC-1 and PFIC-2 was challenging.
  • Late presentation and advanced fibrosis complicated specific subtype identification.
  • Diagnostic criteria included clinical, biochemical, and imaging findings.

Implications:

  • Highlights the importance of considering PFIC in pediatric cholestasis, even with initial misdiagnoses.
  • Emphasizes the need for advanced diagnostic approaches when fibrosis is advanced.
  • Suggests potential for improved early diagnostic markers for PFIC subtypes.