Familial nonhemolytic jaundice with free and conjugated hyperbilirubinemia, elevated serum bile acids, and liver pigmentation

Abstract

We have described an 11-year-old patient with nonhemolytic hyperbilirubinemia, elevated serum bile acids, and liver pigmentation. The Bromsulphalein clearance was not typical of that seen in the Dubin-Johnson syndrome, but there was a secondary rise at 120 min. The abnormalities involving several organic anions and liver histology are compared to those seen in other types of familial nonhemolytic jaundice.