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成人胆管板畸形的影像学特征。

Imaging features of ductal plate malformations in adults.

机构信息

Department of Radiology, Derriford Hospital, Plymouth, UK.

出版信息

Clin Radiol. 2011 Nov;66(11):1086-93. doi: 10.1016/j.crad.2011.05.008. Epub 2011 Aug 15.

Abstract

Ductal plate malformations, also known as fibrocystic liver diseases, are a group of congenital disorders resulting from abnormal embryogenesis of the biliary ductal system. The abnormalities include choledochal cyst, Caroli's disease and Caroli's syndrome, adult autosomal dominant polycystic liver disease, and biliary hamartoma. The hepatic lesions can be associated with renal anomalies such as autosomal recessive polycystic kidney disease (ARPKD), medullary sponge kidney, and nephronophthisis. A clear knowledge of the embryology and pathogenesis of the ductal plate is central to the understanding of the characteristic imaging appearances of these complex disorders. Accurate diagnosis of ductal plate malformations is important to direct appropriate clinical management and prevent misdiagnosis.

摘要

胆管板畸形,又称纤维囊性肝病,是一组因胆管系统胚胎发育异常引起的先天性疾病。其异常包括胆总管囊肿、Caroli 病和 Caroli 综合征、常染色体显性遗传性多囊肝病和胆管错构瘤。肝脏病变可与肾脏异常相关,如常染色体隐性多囊肾病(ARPKD)、髓质海绵肾和肾单位肾痨。了解胆管板的胚胎发生和发病机制对于理解这些复杂疾病的特征性影像学表现至关重要。准确诊断胆管板畸形对于指导适当的临床管理和防止误诊非常重要。

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