Park Byeong Chool, Park Seon Mee, Choi Eun Young, Chae Hee Bok, Yoon Se Jin, Sung Rohyun, Lee Sung Koo
Department of Internal Medicine, Chungbuk National University College of Medicine and Medical Research Institute, Cheongju, Korea.
Korean J Intern Med. 2009 Sep;24(3):270-3. doi: 10.3904/kjim.2009.24.3.270. Epub 2009 Aug 26.
Idiopathic adulthood ductopenia (IAD) is a chronic cholestatic liver disease of unknown etiology characterized by adult onset, an absence of autoantibodies, inflammatory bowel disease, and a loss of interlobular bile ducts. In the present report, a case fulfilling the IAD criteria is described. A 19-year-old man was admitted to the hospital for persistent elevation of transaminases and alkaline phosphatase without clinical symptoms. Viral hepatitis markers and autoantibodies were absent. The patient had a normal extrahepatic biliary tree and had no evidence of inflammatory bowel disease. A liver biopsy specimen showed absence of interlobular bile ducts from 58% of the portal tracts. He was diagnosed with IAD and was treated with ursodeoxycholic acid.
特发性成人胆管减少症（IAD）是一种病因不明的慢性胆汁淤积性肝病，其特征为成年起病、无自身抗体、无炎症性肠病以及小叶间胆管缺失。在本报告中，描述了一例符合IAD标准的病例。一名19岁男性因转氨酶和碱性磷酸酶持续升高入院，无临床症状。病毒肝炎标志物和自身抗体均为阴性。患者肝外胆管树正常，无炎症性肠病证据。肝活检标本显示58%的门管区小叶间胆管缺失。他被诊断为IAD，并接受了熊去氧胆酸治疗。
