Nakanuma Y, Hoso M, Mizuno Y, Unoura M
Second Department of Pathology, Kanazawa University School of Medicine, Japan.
Liver. 1988 Dec;8(6):319-24. doi: 10.1111/j.1600-0676.1988.tb01010.x.
While cholestatic jaundice usually develops in the advanced stages of primary biliary cirrhosis, our series of this disease disclosed that 8 of the 88 cases presented cholestatic jaundice in the early histologic stages (stages 1 and 2). These patients frequently presented esophageal varices (57%) and showed a low incidence of positive mitochondrial antibodies compared to the non-jaundices cases in the same histologic stages. Histologic changes of livers from the jaundiced patients failed to show fundamental differences from those of the non-jaundiced patients, except for the presence of bile plugs and extensive bile duct loss in the former. Deposition of orcein-positive granules and increased hepatic copper content, suggesting prolonged cholestasis, were rather advanced, and inflammatory changes in the portal tracts and piecemeal necrosis were rather mild in the jaundiced patients. These data suggest that cholestatic jaundice occurs in a few patients in the early histologic stages on a biopsy with extensive bile duct loss and features of prolonged cholestasis; it is not clear whether such patients are a small separate cluster in PBC, or not.
虽然胆汁淤积性黄疸通常在原发性胆汁性肝硬化的晚期出现，但我们对该疾病的系列研究发现，88例患者中有8例在组织学早期阶段（1期和2期）出现了胆汁淤积性黄疸。这些患者经常出现食管静脉曲张（57%），与相同组织学阶段的非黄疸病例相比，线粒体抗体阳性的发生率较低。黄疸患者肝脏的组织学变化与非黄疸患者相比，除了前者存在胆汁栓和广泛的胆管缺失外，未显示出根本性差异。orcein阳性颗粒的沉积和肝铜含量的增加表明胆汁淤积时间延长，这种情况相当严重，而黄疸患者门静脉区的炎症变化和碎片状坏死则相当轻微。这些数据表明，在活检时，少数患者在组织学早期阶段出现胆汁淤积性黄疸，伴有广泛的胆管缺失和胆汁淤积时间延长的特征；目前尚不清楚这些患者在原发性胆汁性肝硬化中是否是一个单独的小群体。
