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Cholestatic liver diseases: slow progress in understanding and treating slowly progressive disorders
F P Vleggaar1, N A Van Ooteghem, H R Van Buuren
1Dept. of Hepatogastroenterology, University Hospital Rotterdam, The Netherlands.
Background:
Cholestatic liver diseases are characterized by failure of normal amounts of physiological bile to reach the gastrointestinal tract. Any interference with normal bile flow from the canalicular membrane of the hepatocyte to the distal common bile duct may result in cholestasis.
Methods:
Literature review.
Results:
In primary biliary cirrhosis (PBC), the small intrahepatic bile ducts are destructed, resulting in obstruction of intrahepatic bile flow, whereas extrahepatic and/or intrahepatic biliary strictures block the passage of bile towards the intestine in primary sclerosing cholangitis (PSC). In contrast, the biliary tree is morphologically unaffected in less common cholestatic liver diseases as benign recurrent intrahepatic cholestasis (BRIC) and progressive familiar intrahepatic cholestasis (PFIC1-4). Genetic defects in hepatic canalicular transport mechanisms and bile salt synthesis deficiencies seem to underlie these types of cholestatic disorders.
Conclusion:
Recent advances in understanding and treatment of cholestatic liver diseases may help in better diagnosing and treating the various conditions characterized by cholestasis.