Familial nonhemolytic jaundice with free and conjugated hyperbilirubinemia, elevated serum bile acids, and liver pigmentation.
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.
D001647:Bile Acids and Salts / Q000097:blood*; D001707:Biopsy, Needle; D002648:Child; D005260:Female; D006801:Humans; D006932:Hyperbilirubinemia / Q000150:complications; D007566:Jaundice, Chronic Idiopathic / Q000097:blood / Q000175:diagnosis* / Q000473:pathology; D008099:Liver / Q000032:analysis* / Q000473:pathology; D008297:Male; D010860:Pigments, Biological / Q000032:analysis*; D013448:Sulfobromophthalein
D001647:Bile Acids and Salts; D010860:Pigments, Biological; D013448:Sulfobromophthalein