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

Korean Journal of Pediatrics 2009;52(4):504-507.
Published online April 15, 2009.
Autoimmune hepatitis-primary sclerosing cholangitis overlap syndrome in a 10-year-old girl with ulcerative colitis
Jeana Hong1, Mi Kyoung Song1, Jae Sung Ko1, Gyeong Hoon Kang2, Woo Sun Kim3, Jeong Kee Seo1
1Departments of Pediatrics, Seoul National University College of Medicine, Seoul, Korea
2Departments of Pathology, Seoul National University College of Medicine, Seoul, Korea
3Departments of Radiology, Seoul National University College of Medicine, Seoul, Korea
궤양성 대장염에 동반된 자가면역성 간염-원발성 경화성 담관염의 중복 증후군 1예
홍지나1, 송미경1, 고재성1, 강경훈2, 김우선3, 서정기1
1서울대학교 의과대학 소아과학교실
2서울대학교 의과대학 병리학교실
3서울대학교 의과대학 영상의학과교실
Correspondence: 
Jeong Kee Seo, Email: jkseo@snu.ac.kr
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.
Key Words: Autoimmune Hepatitis, Primary Sclerosing Cholangitis, Overlap Syndrome, Ulcerative Colitis, Inflammatory Bowel Diseases


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