Kraus M R, Scheurlen M
Medizinische Poliklinik der Universität Würzburg.
MMW Fortschr Med. 2004 Jun 3;146(23):34-7.
Prompted by the histomorphological aspect of ductopenia, chronic intrahepatic liver diseases are increasingly being subsumed under the term vanishing bile duct syndrome. Classification by cholestasis syndromes in adults (e.g. primary biliary cirrhosis, primary sclerosing cholangitis) and in the newborn or children (e.g. alphal antitrypsin deficiency, cystic fibrosis) makes good sense. Decisive for the diagnosis are, depending on the disease presenting, a typical constellation of laboratory results, detection of autoantibodies, imaging procedures (e.g. ERC, MRI), liver biopsy where indicated, or suspected drug-induced cholestasis. Byway of treatment, ursodeoxycholic acid, an antibiotic in cholestasis, and liver transplantation in some cases, are possible options. Supportive treatment should be aimed at extrahepatic manifestations of cholestasis (e.g. osteoporosis, vitamin deficiency, pruritus).
受胆管减少的组织形态学特征的启发,慢性肝内疾病越来越多地被归入消失胆管综合征这一术语之下。根据成人胆汁淤积综合征(如原发性胆汁性肝硬化、原发性硬化性胆管炎)以及新生儿或儿童胆汁淤积综合征(如α1抗胰蛋白酶缺乏症、囊性纤维化)进行分类是有意义的。根据所呈现的疾病情况,诊断的决定性因素包括典型的实验室检查结果组合、自身抗体的检测、成像检查(如内镜逆行胰胆管造影、磁共振成像)、必要时的肝活检,或疑似药物性胆汁淤积。在治疗方面,熊去氧胆酸(一种用于胆汁淤积的抗生素)以及在某些情况下进行肝移植是可行的选择。支持性治疗应针对胆汁淤积的肝外表现(如骨质疏松、维生素缺乏、瘙痒)。