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.

JPGN Reports
|July 28, 2026
PubMed

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.

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