All issues > Volume 52(4); 2009

Case Report

Korean J Pediatr. 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 Jn Hong¹, Mi Kyoung MK Song¹, Jae Sung JS Ko¹, Gyeong Hoon GH Kang², Woo Sun WS Kim³, Jeong Kee JK 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

Correspondence Jeong Kee JK 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.

Keywords :Autoimmune Hepatitis, Primary Sclerosing Cholangitis, Overlap Syndrome, Ulcerative Colitis, Inflammatory Bowel Diseases