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Updated: Aug 5, 2026

Isolation of Neonatal Extrahepatic Cholangiocytes
Published on: June 5, 2014
A hepatic enigma: Pediatric presentation of primary biliary cholangitis
Sindhura Kasturi1, Rachel Schenker2, Nick Shillingford3
1Department of Pediatric Gastroenterology, Hepatology and Nutrition Children's Hospital Los Angeles Los Angeles California USA.
Insights
Primary biliary cholangitis (PBC), a rare autoimmune liver disease, can affect children. This case highlights a 14-year-old female diagnosed with PBC, emphasizing the need for pediatric awareness.
Area of Science:
- Hepatology
- Autoimmune Diseases
- Pediatric Gastroenterology
Background:
- Primary biliary cholangitis (PBC) is a chronic autoimmune liver disease.
- It involves the destruction of intrahepatic bile ducts, leading to liver fibrosis and cirrhosis.
- PBC is exceptionally rare in pediatric populations.
Purpose of the Study:
- To report a rare case of PBC in a pediatric patient.
- To increase awareness of PBC as a possibility in children presenting with liver enzyme abnormalities.
- To contribute to the limited literature on pediatric PBC.
Main Methods:
- Case report of a 14-year-old female.
- Clinical presentation with elevated liver enzymes.
- Diagnostic workup for liver disease.
Main Results:
- The patient was diagnosed with Primary Biliary Cholangitis (PBC).
- This diagnosis in a pediatric patient is noteworthy due to the rarity of the condition in this age group.
- The case underscores the importance of considering PBC in the differential diagnosis of pediatric liver conditions.
Conclusions:
- Primary Biliary Cholangitis (PBC) can occur in children.
- Pediatricians and gastroenterologists should consider PBC in the differential diagnosis of unexplained elevated liver enzymes in children.
- Further research and awareness are needed for pediatric PBC.
Abstract:
Primary biliary cholangitis (PBC) is a chronic autoimmune condition characterized by destruction of intrahepatic bile ducts, leading to fibrosis and cirrhosis of the liver. It is an extremely rare pediatric disease with very few pediatric cases reported to date. Here, we report the case of a 14-year-old female who presented with elevated liver enzymes and was subsequently diagnosed with PBC. Our case raises awareness of the possibility of PBC in children.
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