Jaundice in non-cirrhotic primary biliary cirrhosis: the premature ductopenic variant
Jaundice in non-cirrhotic primary biliary cirrhosis: the premature ductopenic variant
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DOI：
10.1136/gut.49.2.276
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发表时间：
2001-08-01
期刊：
影响因子：
24.5
通讯作者：
Hop, WCJ
中科院分区：
文献类型：
--
作者：
Vleggaar, FP;van Buuren, HR;Hop, WCJ
报道了四名患有原发性胆汁性肝硬化 (PBC) 的女性的临床和病理结果，其病程不寻常且迄今尚未得到充分认识。患者出现严重瘙痒和体重减轻，并伴有进行性黄疸胆汁淤积，对熊去氧胆酸和免疫抑制剂等治疗没有反应。在所有病例中，肝脏组织学均显示明显的胆管缺失，但没有明显的纤维化或肝硬化。进一步的诊断研究和重复活检证实不存在肝硬化以及高胆红素血症的其他潜在原因。我们的病例与 101 名非肝硬化 FBC 患者的纤维化与胆管减少关系的比较表明，前者胆管损失的严重程度相对于纤维化的量明显更高。含有小叶间胆管的门静脉三联征的比例为 3%、4%、6% 和 10%，而对照组为 45%（中位数；范围 8.3-100%）（p
The clinical and pathological findings of four females with primary biliary cirrhosis (PBC) with an unusual and hitherto not well recognised course are reported. Patients suffered severe pruritus and weight loss with progressive icteric cholestasis which did not respond to such treatments as ursodeoxycholic acid and immunosuppressives. In all cases liver histology revealed marked bile duct loss without however significant fibrosis or cirrhosis. Further diagnostic studies and repeat biopsies confirmed the absence of liver cirrhosis as well as other potential causes of hyperbilirubinaemia. Comparison of the fibrosis-ductopenia relationship for our cases with that for a group of 101 noncirrhotic FBC patients indicated that in the former the severity of bile duct loss relative to the amount of fibrosis was significantly higher. The proportion of portal triads containing an interlobular bile duct was 3%, 4%, 6%, and 10% compared with 45% (median; range 8.3-100%) for controls (p