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高胆红素血症和胆汁淤积症。

Hyperbilirubinemia and cholestasis.

作者信息

Berk P D, Javitt N B

出版信息

Am J Med. 1978 Feb;64(2):311-26. doi: 10.1016/0002-9343(78)90061-x.

Abstract

Although the morphologist continues to describe cholestasis on the basis of precipitated bile seen on light microscopic sections of the liver or dilated canaliculi with loss of microvilli seen by electron microscopy, the physiologist can distinguish clearly between hyperbilirubinemia and cholestasis. Both bilirubin and bile acids are specifically removed from sinusoidal plasma by the normal hepatocyte and appear in bile in high concentration. Bilirubin conjugation and excretion appear to be governed by hepatocellular mechanisms that are, for the most part, separate from the conjugation and excretion of bile acids. Disturbances in bilirubin transport are recognized by hyperbilirubinemia which represents a number of clinical syndromes that can be classified by the nature of the block in the transport system. Serum bile acids appear to remain normal in hyperbilirubinemic syndromes. By contrast, cholestatic syndromes are characterized by marked bile acidemia with normal to slightly elevated bilirubin levels. Severe cholestasis, because of the marked reduction in bile flow, can however, engender jaundice. Further exploration of these excretory pathways will provide interesting new insights on the numerous cholestatic and hyperbilirubinemic syndromes that occur in nature.

摘要

尽管形态学家仍在根据肝脏光镜切片上所见的胆汁沉淀或电子显微镜下所见的微绒毛缺失的扩张胆小管来描述胆汁淤积,但生理学家能够清楚地区分高胆红素血症和胆汁淤积。正常肝细胞会从肝窦血浆中特异性地清除胆红素和胆汁酸,并使其在胆汁中高浓度出现。胆红素的结合和排泄似乎受肝细胞机制控制,在很大程度上与胆汁酸的结合和排泄机制不同。胆红素转运障碍表现为高胆红素血症,它代表了一些临床综合征,可根据转运系统中阻塞的性质进行分类。在高胆红素血症综合征中,血清胆汁酸似乎保持正常。相比之下,胆汁淤积综合征的特征是胆汁酸血症明显,胆红素水平正常至略有升高。然而,由于胆汁流量显著减少,严重的胆汁淤积会导致黄疸。对这些排泄途径的进一步探索将为自然界中发生的众多胆汁淤积和高胆红素血症综合征提供有趣的新见解。

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