CONGENITAL DISEASES OF INTRAHEPATIC BILE-DUCTS - VARIATIONS ON THE THEME DUCTAL PLATE MALFORMATION
CONGENITAL DISEASES OF INTRAHEPATIC BILE-DUCTS - VARIATIONS ON THE THEME DUCTAL PLATE MALFORMATION
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DOI：
10.1002/hep.1840160434
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发表时间：
1992-10-01
期刊：
影响因子：
13.5
通讯作者：
DESMET, VJ
中科院分区：
文献类型：
--
作者：
DESMET, VJ
先天性肝内胆管疾病可分为两大类：以肝内胆管坏死炎性破坏为特征的疾病和以肝内胆管不同程度扩张并伴有不同程度纤维化为特征的疾病。本综述将强调，在大多数（如果不是全部）这些情况下，胚胎导管板持续或缺乏重塑（所谓的“导管板畸形”[DPM]）是病变的基本前兆(1)。
Congenital diseases of intrahepatic bile ducts (IHBDs) can be divided into two main groups: diseases characterized by necroinflammatory destruction of IHBDs and diseases characterized by a variable degree of ectasia of IHBDs and associated with a variable degree of fibrosis. This review will emphasize that in most-if not all-of these conditions, persistence or lack of remodeling of the embryonic ductal plate (so-called “ductal plate malformation”[DPM]) is an essential precursor of the lesions (1).