Cholestatic liver disease: pathophysiology and therapeutic options
Cholestatic liver disease: pathophysiology and therapeutic options
复制标题
DOI：
10.1034/j.1600-0676.2002.00002.x
复制
发表时间：
2002-04-01
期刊：
影响因子：
--
通讯作者：
Hofmann, AF
中科院分区：
文献类型：
--
作者：
Hofmann, AF
胆汁淤积是由于胆管分泌缺陷或胆管远端胆汁流动受阻所致。在早期原发性胆汁性肝硬化中，由于胆汁的分泌压力或由于某些小管未被阻塞，胆汁分泌继续。完全胆汁淤积时，小肠腔内发生胆汁酸缺乏，导致脂质消化不良和脂溶性维生素吸收不良。也可能发生细菌增殖、细菌移位至淋巴结和内毒素血症，导致急性期反应。胆汁酸在肝细胞中的滞留导致细胞凋亡。胆汁酸在体循环中的积聚导致瘙痒，并可能导致肺和肾中的内皮损伤。早期尝试通过吸附柱上的血液灌流来模拟肝脏排泄功能，但由于各种原因而失败。对白蛋白的体外透析提供了一个现实的，虽然部分模拟肝脏排泄功能的承诺。
Cholestasis results from defective canalicular secretion of bile or obstruction to bile flow distal to the canaliculus. In early primary biliary cirrhosis, bile secretion continues, because of the secretory pressure of bile or because some ductules are not obstructed. With complete cholestasis, a bile acid deficiency occurs in the small intestinal lumen leading to lipid maldigestion and fat-soluble vitamin malabsorption. Bacterial proliferation, bacterial translocation to lymph nodes and endotoxemia may also occur leading to an acute phase reaction. Retention of bile acids in the hepatocyte leads to apoptosis. Accumulation of bile acids in the systemic circulation leads to pruritus, and may contribute to endothelial injury in the lungs and kidney. Early attempts to mimic hepatic excretory function by hemoperfusion over adsorbent columns were unsuccessful for a variety of reasons. Extracorporeal dialysis against albumin offers promise of a realistic albeit partial simulation of hepatic excretory function.