Constitutional hyperbilirubinemia with unconjugated bilirubin in the serum and pigment deposition in the liver

Hepatic Aspects of Bilirubin Metabolism

“…Consistent with this interpretation is the report of a patient with Dubin-Johnson syndrome who developed viral hepatitis at which time hepatic pigment w a s released from damaged parenchymal cells, engulfed by phagocytic cells, and slowly reaccumulated in regenerating par enchymal liver cells (162). The presence of normal lipofuscin in some livers is probably responsible for reports of hybrids of the variolls syndromes rep resented as cases of unconjugated hyperbilirubinemia with hepatic pigmen tation (163,164) and hepatic pigmentation without hyperbilirubinemia (165) Based on staining reactions and physical-chemical studies, Wegmann and associates (166) suggest that the pigment in Dubin-Johnson syndrome is an atypical melanin. Further knowledge of the structure of the pigment has come from studies of mutant Corriedale sheep which have a functional and morphologic defect identical to that of the Dubin-Johnson sY1'ldrome in man (167,168).…”