New Concepts in Biliary Cirrhosis
New Concepts in Biliary Cirrhosis
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胆汁性肝硬化的新概念
DOI：
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发表时间：
1987
期刊：
影响因子：
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通讯作者：
J. Ludwig
中科院分区：
文献类型：
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作者：
J. Ludwig
传统上，只有两种类型的胆道肝硬化被认可：原发性和次要。原发性胆道肝硬化（PBC）是一种明确定义的慢性进行性肝脏疾病，其特征是分段肉芽肿的肉芽肿室内囊肿和囊状胆管以及一组临床和实验室特征。管道损伤导致门户和间隔纤维化，最终导致了真正的胆道C i r r h o〜i s。 〜“次生胆道肝硬化”一词描述了长期阻塞肝外或脊椎外胆管的后期表现。此外，该名称被用作非主要类型的任何类型的胆道肝硬化的集体名称。目前，首选术语是针对大型管道阻塞而导致的状况的“阻塞性胆道肝硬化”，而没有其他形容词的“胆道肝硬化”，其余的非主要形式的胆道肝硬化（表1）。近年来，详细的形态学研究表明，小型胆管的炎性非支持性破坏并不是PBC综合征的独家表现。其他胆道疾病具有这一重要的致病特征。因此，已经识别出几种肝病不是PBC，而是形态学上的肝脏疾病比经典或大型导管阻塞性胆道肝硬化更为类似于PBC。在本文中，我试图简要描述这些条件，并将它们纳入胆道肝硬化的致病分类中，以大型导管和小型导管疾病的概念（表2和表3）。为了提高演示文稿的清晰度，本文末尾提供了词汇表。
Traditionally, only two types of biliary cirrhosis have been recognized: primary and secondary. Primary biliary cirrhosis (PBC) is a well-defined chronic progressive liver disease, characterized by segmental granulomatous destruction of interlobular and septal bile ducts as well as a characteristic set of clinical and laboratory features.' The duct damage leads to portal and septal fibrosis and, ultimately, true biliary c i r r h o ~ i s . ~ The term "secondary biliary cirrhosis" describes the late manifestations of prolonged blockage of extrahepatic or perihilar intrahepatic bile ducts. In addition, the name is used as a collective designation for any type of biliary cirrhosis that is not primary. Currently, the preferred terms are "obstructive biliary cirrhosis" for the condition resulting from large-duct blockage, and "biliary cirrhosis," without another adjective, for the remaining nonprimary forms of biliary cirrhosis (Table 1). In recent years, detailed morphologic studies have shown that inflammatory nonsuppurative destruction of small bile ducts is not an exclusive manifestation of the syndrome of PBC; other biliary diseases share this important pathogenetic feature. Thus, several liver diseases have been recognized that are not PBC but morphogenetically resemble PBC more closely than classic or large-duct obstructive biliary cirrhosis. In this article, I have attempted to describe briefly these conditions and to incorporate them in a pathogenetic classification of biliary cirrhosis, based on the concept of large-duct and small-duct disease (Tables 2 and 3). In order to enhance clarity of the presentation, a glossary has been provided at the end of the article.
肝硬化是移植物抗宿主病的结果。
影响因子：
29.4
作者：
Knapp,AB;Crawford,JM;Rappeport,JM;Gollan,JL
通讯作者：
Gollan,JL