[Intrahepatic cholestasis caused by biochemical errors of bile acids. Part I --Nosographic, pathogenetic, diagnostic...

Intrahepatic cholestasis occurs in certain conditions characterized by a biochemical error of bile acid metabolism, resulting from a disorder of the hepatic canalicular system responsible for synthesis or secretion of the bile acids. As regards the pathogenesis of these "primary" forms of cholestasis, it must be remembered that cholestasis represents the outcome of various factors capable of interfering with the mechanism of bile flow. Therefore the factors known to be involved in cholestasis, such as the metabolic steps in bile acid metabolism, the cytoplasmic membrane, the mitochondria, the cytoskeleton of the liver cell, the intercellular junctions, the physicochemical state of the canalicular bile, are discussed briefly. The diagnostic and clinical aspects of cholestasis with reference to the clinical symptoms, laboratory findings and to role of liver biopsy are synthesized, and the essential criteria for a methodological approach to cholestasis are proposed.