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Primary sclerosing cholangitis associated with immunodeficiency
American Journal of Diseases of Children (1960)
|February 1, 1983
Summary
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.
- PSC should be included in the differential diagnosis for pediatric cholestatic jaundice.