摘要
Vanishing bile duct syndromes （VBDS） are characterized by progressive loss of small intrahepatic ducts caused by a variety of different diseases leading to chronic cholestasis, cirrhosis, and premature death from liver failure. The majority of adult patients with VBDS suffer from primary biliary cirrhosis （PBC） and primary sclerosing cholangitis （PSC）. Ursodeoxycholic acid （UDCA）, a hydrophilic dihydroxy bile acid, is the only drug currently approved for the treatment of patients with PBC, and anticholestatic effects have been reported for several other cholestatic syndromes. Several potential mechanisms of action of UDCA have been proposed including stimulation of hepatobiliary secretion, inhibition of apoptosis and protection of cholangiocytes against toxic effects of hydrophobic bile acids.
消失的胆汁管症候群(VBDS ) 被从肝失败导致长期的胆汁郁积，肝硬化，和早熟的死亡的许多不同疾病引起的小肝内管的进步损失描绘。有 VBDS 的成年病人的多数受不了主要胆汁性肝硬变(PBC ) 和主要致硬化的胆管炎(PSC ) 。Ursodeoxycholic 酸(UDCA ) ，吸水的 dihydroxy 胆汁酸，唯一的药当前被同意因为有 PBC 的病人的治疗，和 anticholestatic 效果为几另外的胆汁郁积的症候群被报导了。UDCA 的行动的几潜在的机制包括肝胆管分泌物的刺激被建议了， apoptosis 的抑制和对恐水病的胆汁酸的有毒的效果的 cholangiocytes 的保护。
