Lipids and lipid-activated vitamins in chronic cholestatic diseases.
Lipids and lipid-activated vitamins in chronic cholestatic diseases.
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DOI：
10.1016/s1089-3261(05)70013-1
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发表时间：
1998-05-01
影响因子：
5.1
通讯作者：
Kowdley, K V
中科院分区：
文献类型：
--
作者：
Kowdley, K V
原发性胆汁性肝硬化、原发性硬化性胆管炎和自身免疫性胆管病是原因不明的胆汁淤积性肝病。中小胆管（在原发性胆汁性肝硬化和自身免疫性胆管病中）和大胆管（在原发性硬化性胆管炎中）的破坏导致进行性胆汁淤积、肝功能衰竭和终末期肝病。在原发性胆汁性肝硬化中已经描述了多种脂质代谢异常，范围从血清脂质水平和脂蛋白亚群的改变到胆固醇代谢紊乱。在这些胆汁淤积性肝病中，进行性胆汁淤积和因此小肠胆汁酸浓度降低也可导致脂肪和脂溶性维生素吸收受损，从而导致脂肪过多和维生素A、D、E和K缺乏。本文重点介绍原发性胆汁性肝硬化和原发性硬化性胆管炎的脂质代谢异常，以及这些疾病中的脂质激活维生素缺乏症。
Primary biliary cirrhosis, primary sclerosing cholangitis and autoimmune cholangiopathy are cholestatic liver diseases of unknown cause. Destruction of small to medium bile ducts (in primary biliary cirrhosis and autoimmune cholangiopathy) and large bile ducts (in primary sclerosing cholangitis) leads to progressive cholestasis, liver failure and end-stage liver disease. A variety of abnormalities in lipid metabolism have been described in primary biliary cirrhosis, and range from alterations in serum lipid levels and lipoprotein subsets to deranged metabolism of cholesterol. Progressive cholestasis and, consequently, decreased small intestinal bile acid concentrations in these cholestatic liver disease can also lead to impaired absorption of fats and fat-soluble vitamins, resulting in steatorrhea and deficiencies in vitamins A, D, E, and K. This article focuses on abnormalities in lipid metabolism in primary biliary cirrhosis and primary sclerosing cholangitis, and on lipid-activated vitamin deficiencies in these disorders.