Characteristic differences according to the cirrhotic pattern of advanced primary biliary cirrhosis: Macronodular cirrhosis indicates slow progression
Characteristic differences according to the cirrhotic pattern of advanced primary biliary cirrhosis: Macronodular cirrhosis indicates slow progression
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DOI：
10.1016/j.hepres.2006.07.008
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发表时间：
2006-11-01
影响因子：
4.2
通讯作者：
Tsuneyoshi, Masazumi
中科院分区：
文献类型：
--
作者：
Aishima, Shinichi;Kuroda, Yousuke;Tsuneyoshi, Masazumi
对晚期原发性胆汁性肝硬化（PBC）进行临床病理学评估，对阐明其进展机制具有重要意义。将26例原发性PBC按肝硬化类型分为非肝硬化4例、大结节型肝硬化4例、混合结节型肝硬化6例和小结节型肝硬化12例，比较其临床和形态学特点。此外，还分析了肝内胆管的保存程度和其他组织学特征。大结节性肝硬化活体肝移植（LDLT）患者的年龄明显大于小结节性肝硬化患者。大结节性肝硬化的临床表现和LDLT之间的平均持续时间明显长于小结节性肝硬化。非癫痫组显示临床表现和LDLT之间的持续时间较短。缩小肝体积与标准肝体积的比值（ELV/SLV）显示大结节型肝硬化较其他三型肝硬化更易发生萎缩性改变。在大结节性肝硬化的病例中，每组小于50 μ m的残余肝内胆管的密度显著高于小结节性肝硬化的病例。因此，晚期PBC的不同病理类型与疾病进展及胆管消失程度有关。大结节性胆管炎患者年龄较大，病程较长，但胆管丢失较少。我们认为PBC的大结节性肝硬化和小结节性肝硬化是不同类型的PBC。(C)2006年由Elsevier爱尔兰有限公司出版。
It is important to evaluate advanced primary biliary cirrhosis (PBC) clinicopathologically to clarify its progressive mechanism. According to the cirrhotic pattern, 26 cases of explanted PBC were classified into non-cirrhotic (n = 4), macronodular (n = 4), mixed nodular (n = 6), and micronodular cirrhosis (n = 12), to compare their clinical and morphological features. In addition, the degree of preserved intrahepatic bile ducts and other histologic features were analyzed. Patients at living donor liver transplantation (LDLT) in the macronodular cirrhosis were significantly older than those in the micronodular cirrhosis. The mean duration between clinical presentation and LDLT in the macronodular cirrhosis was significantly longer than in the micronodular cirrhosis. The non-cirrhotic group showed a short duration between clinical presentation and LDLT. The ratio of explanted liver volume to standard liver volume (ELV/SLV) indicates that macronodular cirrhosis revealed more atrophic change than that in the other three types. The density of remnant intrahepatic bile ducts of less than 50 mu m per group in cases of macronodular cirrhosis was significantly higher than that in cases of micronodular cirrhosis. Therefore, different cirrhotic patterns of advanced PBC were correlated with the disease progression and the degree of bile duct disappearance. The macronodular cirrhotic patients were older, had a longer disease course, yet had less bile duct loss. We suggest that macronodular cirrhosis and micronodular cirrhosis of PBC are different type of PBC. (C) 2006 Published by Elsevier Ireland Ltd.