Cholestatic liver diseases: Slow progress in understanding and treating slowly progressive disorders

Background: Cholestatic liver diseases are characterized by failure of norm al amounts of physiological bile to reach the gastrointestinal tract. Any i nterference with normal bile Row from the canalicular membrane of the hepat ocyte to the distal common bile duct may result in cholestasis. Methods: Li terature review. Results: In primary biliary cirrhosis (PBC), the small int rahepatic bile ducts are destructed, resulting in obstruction of intrahepat ic bile flow, whereas extrahepatic and/or intrahepatic biliary strictures b lock the passage of bile towards the intestine in primary sclerosing cholan gitis (PSC). In contrast, the biliary tree is morphologically unaffected in less common cholestatic liver diseases as benign recurrent intrahepatic ch olestasis (BRIC) and progressive familiar intrahepatic cholestasis (PFICl-4 ). Genetic defects in hepatic canalicular transport mechanisms and bile sal t synthesis deficiencies seem to underlie these types of cholestatic disord ers. Conclusion: Recent advances in understanding and treatment of cholesta tic liver diseases may help in better diagnosing and treating the various c onditions characterized by cholestasis.