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Related Experiment Videos

Sclerosing cholangitis in children

D Debray1, D Pariente, E Urvoas

  • 1Service d'Hépatologie Pédiatrique, Hôpital de Bicêtre, Le Kremlin-Bicêtre, France.

The Journal of Pediatrics
|January 1, 1994
PubMed
Summary
This summary is machine-generated.

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Sclerosing cholangitis (SC) in children often presents with jaundice and liver enlargement. Prognosis is poor, but liver transplantation offers a chance for survival, especially when SC is not linked to severe immunodeficiency.

Area of Science:

  • Pediatric Gastroenterology
  • Hepatology
  • Immunology

Background:

  • Sclerosing cholangitis (SC) is a rare, chronic cholestatic liver disease affecting children.
  • Early diagnosis and understanding of associated conditions are crucial for management.

Purpose of the Study:

  • To describe the clinical characteristics, histopathology, and outcomes of pediatric sclerosing cholangitis.
  • To evaluate the role of liver transplantation in managing severe cases.

Main Methods:

  • Retrospective analysis of 56 children diagnosed with SC between 1972 and 1992.
  • Clinical data, laboratory findings, histopathology, cholangiography, and treatment outcomes were reviewed.

Main Results:

  • SC presented with neonatal cholestasis or later onset associated with conditions like histiocytosis X or immunodeficiency.

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  • Most children developed biliary cirrhosis; median survival was 10 years.
  • Liver transplantation improved survival in 11 of 15 recipients.

    • Conclusions:

    • SC should be suspected in children with chronic cholestatic disease and elevated gamma-glutamyltransferase.
    • Prognosis is generally poor, but liver transplantation is a viable option for select patients.