Korean J Pediatr.  2009 Apr;52(4):504-507. 10.3345/kjp.2009.52.4.504.

Autoimmune hepatitis-primary sclerosing cholangitis overlap syndrome in a 10-year-old girl with ulcerative colitis

Affiliations
  • 1Department of Pediatrics, Seoul National University College of Medicine, Seoul, Korea. jkseo@snu.ac.kr
  • 2Department of Pathology, Seoul National University College of Medicine, Seoul, Korea.
  • 3Department of Radiology, Seoul National University College of Medicine, Seoul, Korea.

Abstract

Autoimmune hepatitis (AIH), primary sclerosing cholangitis (PSC), and primary biliary cirrhosis (PBC) are chronic autoimmune liver diseases. Overlap syndrome is defined as a condition in which the clinical, biochemical, and histological features of these autoimmune diseases are overlapped. Thus, it is difficult to appreciate overlap syndrome as an actual diagnostic entity. Only a few cases of the overlap syndrome of AIH and PSC have been reported, especially in children. Moreover, PSC is known to be the most frequent liver disorder associated with inflammatory bowel diseases such as ulcerative colitis. We report one case of AIH-PSC overlap syndrome in a child who was diagnosed as having ulcerative colitis.

Keyword

Autoimmune Hepatitis; Primary Sclerosing Cholangitis; Overlap Syndrome; Ulcerative Colitis; Inflammatory Bowel Diseases

MeSH Terms

Autoimmune Diseases
Child
Cholangitis, Sclerosing
Colitis, Ulcerative
Hepatitis, Autoimmune
Humans
Inflammatory Bowel Diseases
Liver
Liver Cirrhosis, Biliary
Liver Diseases
Ulcer
Cholangitis, Sclerosing
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