Related Experiment Video

Updated: Jul 11, 2025

A Mouse Model of Chronic Liver Fibrosis for the Study of Biliary Atresia
09:12

A Mouse Model of Chronic Liver Fibrosis for the Study of Biliary Atresia

Published on: February 3, 2023

2.5K

Congenital Hepatic Fibrosis in a 2-Year-Old Child Presenting with Fever of Unknown Origin

Michael P Penfold1, Wentiirim B Annankra1, Nathan C Hull1

  • 1Mayo Clinic, Rochester, MN, USA.

Case Reports in Pediatrics
|November 10, 2023
PubMed

Insights

Congenital hepatic fibrosis is a rare genetic disorder causing bile duct issues and liver scarring. Early diagnosis in children is crucial for managing complications like gastrointestinal bleeding.

Area of Science:

  • Pediatric Gastroenterology
  • Hepatology
  • Medical Genetics

Background:

  • Congenital hepatic fibrosis (CHF) is a rare, inherited condition.
  • It stems from abnormal development of the bile duct's "ductal plate."
  • CHF leads to liver scarring (fibrosis) and bile duct cysts.

Observation:

  • A 2-year-old child presented with an 8-day fever.
  • No significant past medical history or physical findings were noted.
  • This presentation prompted investigation into underlying conditions.

Findings:

  • The child was diagnosed with congenital hepatic fibrosis.
  • This rare disease involves bile duct malformation and liver fibrosis.
  • Progressive fibrosis can cause portal hypertension and gastrointestinal bleeding.

Implications:

  • Early diagnosis of CHF in children is vital.
  • Prompt management can mitigate risks of portal hypertension and hemorrhage.
  • Understanding ductal plate malformation is key to treating this condition.

More Related Videos

Isolation of Neonatal Extrahepatic Cholangiocytes07:54

Isolation of Neonatal Extrahepatic Cholangiocytes

Published on: June 5, 2014

10.3K
Author Spotlight: Advancing Hepatic Fibrosis Diagnosis Using Magnetic Resonance Elastography and AI06:09

Author Spotlight: Advancing Hepatic Fibrosis Diagnosis Using Magnetic Resonance Elastography and AI

Published on: July 21, 2023

1.2K

Related Concept Videos

Increased Body Temperature01:25

Increased Body Temperature

A body temperature above  38°C  (100.4 °F) is known as fever or pyrexia, and a person with fever is termed 'febrile.' Typically, the hypothalamus, a part of the brain that acts as the body's thermostat, regulates body temperature through a thermoregulatory setpoint. It receives signals from cold and warm thermal receptors throughout the body and adjusts the body's temperature accordingly. Fever occurs when this hypothalamic setpoint is altered, usually in...
685
Types of Fever01:25

Types of Fever

Fever can be triggered by several factors, including infections, nervous system disorders, certain cancers, blood diseases like leukemia, embolism, thrombosis, heatstroke, dehydration, surgical trauma, crushing injuries, and allergic reactions.
Here are the different types of fever:
380
Patterns of Fever01:26

Patterns of Fever

Before understanding the types and patterns of fever, it is essential to know its phases.
2.6K
Diseases of the Liver and Gallbladder01:26

Diseases of the Liver and Gallbladder

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.
Cirrhosis is characterized by the scarring of hepatic lobules in the liver, which are replaced by fibrous tissue, affecting the liver's normal functioning. NAFLD, on the other hand, is caused by an excessive build-up of fat in the liver, not...
579
Liver Regeneration01:24

Liver Regeneration

The liver is an important organ in vertebrates that plays an essential role in metabolism. It is also responsible for storing and redistributing nutrients such as carbohydrates, fats, and vitamins in the body. Additionally, the liver releases bile salts which are critical for digesting food and eliminating toxic metabolites from the body.
Cells of Liver
The liver comprises four major types of cells— hepatocytes, stellate, Kupffer, and sinusoidal endothelial cells. The hepatocytes are...
3.3K
Appendicitis-I: Introduction01:22

Appendicitis-I: Introduction

The appendix, a small, narrow, blind tube extending from the inferior part of the cecum, is widely regarded as a vestigial organ, having lost much of its original function through evolution. Despite its diminished role, the appendix can become inflamed, a condition known as appendicitis.
Etiology: Appendicitis can arise from various causes, primarily rooted in the obstruction of the appendix lumen. Factors contributing to this obstruction include fecal accumulation, lymphoid hyperplasia and, in...
188