Zhang Xiao-Chen, Wang Di, Li Xiang, Hu Yu-Lin, Wang Chong
Department of Hepatology, The First Hospital of Jilin University, Changchun 130021, Jilin Province, China.
World J Clin Cases. 2020 Dec 26;8(24):6524-6528. doi: 10.12998/wjcc.v8.i24.6524.
Idiopathic adulthood ductopenia (IAD) is a chronic cholestatic liver disease of unknown etiology that usually presents as unexplained jaundice. It is characterized by adult onset, lack of autoantibodies, inflammatory bowel disease and loss of interlobular bile ducts.
This case presents a 27-year-old woman with elevation of transaminases and alkaline phosphatase without clinical symptoms. Five years ago, the patient had abnormal transaminases but no cholestasis. Three months before admission, physical examination revealed an increase in transaminases. Oral hepatoprotective drugs did not show any significant improvement, and she was admitted to hospital for further diagnosis and treatment. Liver biopsy confirmed IAD. After about 2 wk of ursodeoxycholic acid treatment, serological and histological examination showed a significant response.
IAD is a manifestation of cholestasis, but also may be an abnormal increase in transaminase in the early stage.
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，通常表现为不明原因的黄疸。其特征为成人起病、缺乏自身抗体、无炎症性肠病以及小叶间胆管丧失。
本病例为一名27岁女性，转氨酶和碱性磷酸酶升高，但无临床症状。5年前，患者转氨酶异常，但无胆汁淤积。入院前3个月，体格检查发现转氨酶升高。口服保肝药物未见明显改善，遂入院进一步诊治。肝脏活检确诊为IAD。经熊去氧胆酸治疗约2周后，血清学和组织学检查显示有显著反应。
IAD是胆汁淤积的一种表现，但早期也可能表现为转氨酶异常升高。
