Hepatic Aspects of Bilirubin Metabolism

Liver ultrastructure in Gilbert's syndrome.

“…A similar but less severe enzyme defect can be demonstrated in some cases of Gilbert's syndrome (Black and Billing, 1969). According to Arias (1962;1966), these cases have unconjugated bilirubin levels in the range 8-20 mg/100 ml serum, which is in excess of the amounts found in all our patients at the time of liver biopsy. Milder degrees of hyperbilirubinaemia may occur in association with a variety of infections and metabolic disorders (Levine and Klatskin, 1964), but all our patients appeared healthy apart from the episodes of jaundice.…”

Rotor’s Syndrome: A Family Study*

“…As was indicated in the original report of Rotor and associates, it is hoped that future investigations may provide more definite clues to the exact nature of the pathophysiology of this disorder. Much is still not known about the processes involved in bilirubin transport 15,16 from plasma to bile, and it is possible that further studies of patients with Rotor's syndrome should also prove helpful in elucidating those poorly understood mechanisms.…”

How we have learned about the complexity of physiology, pathobiology and pharmacology of bile acids and biliary secretion

“…A complete view of biliary physiology also needs to consider the participation of cholangiocytes in bile formation and the knowledge of mechanisms of bile secretion of other important endogenous compounds, such as cholesterol, bilirubin, glutathione and xenobiotics, such as drugs and toxins. We owe many important contributions in these fields to LaRusso [42] , Ballatori [43] , Arias [44] , Keppler [17] , Sugiyama [19] , Wolkoff [45] , Oude Elferink [46] , Meijer [46] , Kuipers [46] , Jansen [46] , Groen [46] , Groothuis [46] , Ostrow [47] , Fevery [48] , Coleman [49] , Berenson [50] , Vore [51] and many others. As I said this editorial letter of gratitude is highly incomplete, but it would be even more so without mentioning the appreciation of many hepatologists to the supporting role of Dr. Falk and the Falk Foundation e.V.…”