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Related Concept Videos

Bile01:19

Bile

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Bile is a crucial bodily fluid, characterized by its yellow-green color and alkaline nature. Produced in the liver, it is transported through the common hepatic duct into either the cystic duct, leading to the gallbladder, or directly into the common bile duct. The flow of bile is regulated by the sphincter of Oddi located at the entrance of the duodenum. When this sphincter is closed, bile is redirected to the gallbladder for storage and concentration.
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Amino acids03:42

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Amino acids are the monomers that comprise proteins. Each amino acid has the same fundamental structure, which consists of a central carbon atom, or the alpha (α) carbon, bonded to an amino group (NH2), a carboxyl group (COOH), and to a hydrogen atom. Every amino acid also has another atom or group of atoms bonded to the central atom known as the R group. There are 20 common amino acids present in proteins, each with a different R group. Variation in the amino acid sequence is responsible for...
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Transfer RNA Synthesis02:36

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Related Experiment Video

Updated: Jan 29, 2026

Using Multi-fluorinated Bile Acids and In Vivo Magnetic Resonance Imaging to Measure Bile Acid Transport
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Using Multi-fluorinated Bile Acids and In Vivo Magnetic Resonance Imaging to Measure Bile Acid Transport

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Bile Acid Synthesis Defect and Hyperinsulinism.

Michael Rogers1, Francisco Sylvester1, Steven Lichtman1

  • 1Department of Pediatric Gastroenterology, University of North Carolina Children's Hospital, Chapel Hill, NC.

ACG Case Reports Journal
|February 19, 2019
PubMed
Summary
This summary is machine-generated.

Rare bile acid synthesis defects can cause liver failure in infants. This case highlights a potential link between this liver dysfunction and prolonged neonatal hyperinsulism (PNH), suggesting PNH may arise from cholestatic stress.

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Area of Science:

  • Biochemistry
  • Pediatric Gastroenterology
  • Neonatology

Background:

  • Congenital defects in bile acid synthesis (BASD) are rare genetic disorders.
  • These defects lead to progressive liver dysfunction and failure.
  • Prolonged neonatal hyperinsulism (PNH) is a distinct condition characterized by persistent hypoglycemia due to stress.

Observation:

  • A 4-month-old infant presented with severe liver failure attributed to a bile acid synthesis defect.
  • The infant also developed prolonged neonatal hyperinsulism (PNH).
  • Liver failure symptoms resolved with oral cholic acid treatment.

Findings:

  • Treatment with oral cholic acid successfully resolved the infant's liver failure.
  • The prolonged neonatal hyperinsulism (PNH) observed in the infant resolved gradually over time.
  • This case suggests a potential association between cholestatic liver failure and the development of PNH.

Implications:

  • The findings suggest that cholestatic liver failure may act as a specific stressor contributing to the development of PNH.
  • Understanding this relationship could help identify infants at higher risk for PNH.
  • Further research into the interplay between liver dysfunction and metabolic stress in neonates is warranted.