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Related Concept Videos

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...
Pharmacokinetics in Pediatric Patients: Overview and Drug Absorption01:23

Pharmacokinetics in Pediatric Patients: Overview and Drug Absorption

Understanding the physiological differences in the pediatric population is crucial for effective pharmacotherapy. Neonates, infants, and children exhibit significant variations in gastric pH, gastric emptying time, intestinal transit time, and biliary function. These variations profoundly affect oral drug absorption, necessitating a nuanced approach to pediatric dosing.Neonates present with a unique physiological profile, having a gastric pH greater than 4 and faster and more irregular gastric...
Pharmacokinetics in Pediatric Patients: Drug Metabolism01:24

Pharmacokinetics in Pediatric Patients: Drug Metabolism

In pediatric care, understanding the nuances of hepatic drug metabolism is crucial, as it significantly differs from that of adults. This divergence is primarily due to the developmental stage of drug-metabolizing enzymes, which affects how medications are processed in the body. In neonates, for instance, the activity of Phase I enzymes—critical for the initial breakdown of drugs—is markedly reduced, functioning at just 20–40% of the levels seen in adults. This reduction poses a challenge in...
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...
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 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...

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Related Experiment Video

Updated: Jul 15, 2026

Partial Bile Duct Ligation in the Mouse: A Controlled Model of Localized Obstructive Cholestasis
04:38

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Published on: March 28, 2018

Pediatric-onset primary biliary cirrhosis.

Yaser Dahlan1, Leslie Smith, Doug Simmonds

  • 1Division of Gastroenterology, Department of Medicine, University of Alberta, 205 College Plaza, 8215-112 Street, Edmonton, Alberta, Canada T6G 2C7.

Gastroenterology
|November 5, 2003
PubMed
Summary

Primary biliary cirrhosis (PBC), an autoimmune liver disease, is rarely seen in children. This report details two pediatric cases of PBC, highlighting the importance of early detection and diagnosis in this age group.

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Area of Science:

  • Hepatology
  • Autoimmune Diseases
  • Pediatric Gastroenterology

Background:

  • Primary biliary cirrhosis (PBC) is a chronic autoimmune liver disease typically diagnosed in middle-aged women.
  • Childhood-onset PBC is exceedingly rare, with limited reported cases in the pediatric population.

Observation:

  • Two adolescent females, aged 16 and 15, were diagnosed with biopsy-confirmed, antimitochondrial antibody (AMA)-positive PBC.
  • The first patient experienced progressive cholestasis, pruritus, neuropathy, and weight loss, ultimately requiring liver transplantation.
  • The second patient showed near-normalization of liver enzymes with ursodeoxycholic acid treatment.

Findings:

  • This study presents the first reported cases of pediatric-onset, AMA-positive PBC.
  • The findings suggest that PBC can manifest in adolescents, challenging the traditional view of its typical demographic.
  • Genetic predisposition may play a role, as the first patient's mother had PBC/autoimmune overlap syndrome.

Implications:

  • Increased awareness and diagnostic vigilance for early-onset PBC in pediatric patients are crucial.
  • Prompt diagnosis and treatment may alter the disease course in pediatric PBC.
  • Further research is needed to understand the pathogenesis and long-term outcomes of childhood PBC.