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Related Experiment Videos

Primary sclerosing cholangitis associated with immunodeficiency.

Y Naveh, H Mendelsohn, G Spira

    American Journal of Diseases of Children (1960)
    |February 1, 1983
    PubMed
    Summary
    This summary is machine-generated.

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    Primary sclerosing cholangitis (PSC) can occur in immunodeficient children. This case highlights the link between immunodeficiency and PSC, suggesting it as a potential cause.

    Area of Science:

    • Pediatric Gastroenterology
    • Immunology

    Background:

    • Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease.
    • Immunodeficiency is not typically associated with PSC, but warrants further investigation.

    Observation:

    • An infant presented with poor weight gain, steatorrhea, and hepatomegaly.
    • Diagnostic workup revealed cholestatic jaundice, dilated bile ducts, and hepatocellular disease.

    Findings:

    • Pathologic and roentgenographic findings confirmed primary sclerosing cholangitis (PSC).
    • The patient was diagnosed with an underlying immunodeficiency disorder.

    Implications:

    • Immunodeficiency may play a role in the pathogenesis of PSC in select patients.
    • Consider screening immunodeficient children and patients with ulcerative colitis for PSC.

    Related Experiment Videos

  • PSC should be included in the differential diagnosis for pediatric cholestatic jaundice.