Cholestasis in infancy. Status report and conceptual approach.

This review is concerned with the disturbances in bile flow that occur 
early in life. Although cholestasis is defined physiologically, it can be 
considered to have morphological features. Morphological cholestasis is the presence of 
bile pigments in histopathological sections of the liver and is most 
easily recognized as bile plugs or thrombi in bile canaliculi. Physiological 
cholestasis, a reduction in bile flow, is inferred from the finding 
of an elevated serum bile acid. This inference is derived from 
the known positive correlation between canalicular bile flow and bile acid 
excretion that has been found to occur in all species this 
far examined, including man. An increase in the amount of bile 
acid in plasma implies a reduction in the hepatic transport of 
the primary conjugated bile acids of man and therefore a decrease 
in associated canalicular bile flow. Classifications of cholestatic syndromes, functional abnormalities, 
and therapy for cholestasis syndromes are discussed. 