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除原发性胆汁性肝硬化外的慢性肝病中,肝内胆管树的 florid 导管病变和广泛胆管丢失。

Florid duct lesions and extensive bile duct loss of the intrahepatic biliary tree in chronic liver diseases other than primary biliary cirrhosis.

作者信息

Nakanuma Y, Ohta G, Takeshita H, Yamazaki Y, Doishita K, Shimizu M

出版信息

Acta Pathol Jpn. 1983 Nov;33(6):1095-104. doi: 10.1111/j.1440-1827.1983.tb02155.x.

Abstract

Intrahepatic biliary tree with either florid duct lesions or a moderate to severe degree of the duct loss in four livers with chronic hepatic diseases other than primary biliary cirrhosis were studied with histometric and serial section observations. Florid duct lesions, distributed segmentally in the liver, were found in one case with incomplete septal cirrhosis and one case with idiopathic portal hypertension. The florid duct lesions including marked plasma cell infiltration and occasional periductal granulomas, were not associated with any bile duct loss in the two cases. The duct lesions were reversible in one case during a long clinical course. On the other hand, a moderate to severe bile duct loss with biliary epithelial degeneration and necrosis was associated with no or little periductal inflammatory cell infiltration in one other case with chronic intrahepatic cholestasis, probably drug-induced, and in one case with idiopathic portal hypertension. Although florid duct lesions and bile duct loss were important diagnostic features of primary biliary cirrhosis, one of them was observed to develop independently in severely diseased livers, not consistent with a diagnosis of primary biliary cirrhosis, sclerosing cholangitis or intrahepatic bile duct paucity syndrome.

摘要

对4例患有除原发性胆汁性肝硬化以外的慢性肝病的肝脏进行了组织测量和连续切片观察,研究其肝内胆管树,这些肝脏存在明显的胆管病变或中度至重度胆管缺失。在1例不完全性间隔性肝硬化和1例特发性门静脉高压症患者中发现了呈节段性分布于肝脏的明显胆管病变。在这2例中,包括显著浆细胞浸润和偶见的胆管周围肉芽肿的明显胆管病变,均未伴有任何胆管缺失。在一个较长的临床病程中,1例患者的胆管病变是可逆的。另一方面,在另1例可能由药物引起的慢性肝内胆汁淤积患者以及1例特发性门静脉高压症患者中,中度至重度胆管缺失伴胆管上皮变性和坏死,且胆管周围炎性细胞浸润极少或无。虽然明显胆管病变和胆管缺失是原发性胆汁性肝硬化的重要诊断特征,但在病情严重的肝脏中,其中之一可独立出现,这与原发性胆汁性肝硬化、硬化性胆管炎或肝内胆管稀少综合征的诊断不符。

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