Summary
Plasma clearance studies with Indocyanine Green (ICG) and Bromsulfophthalein (BSP) were performed on four patients with chronic conjugated hyperbilirubinemia with or without apparent brown pigments in the liver cells, and free from structural hepatic diseases.
An abnormal clearance pattern, characterized by reduced hepatic ICG and BSP clearances, was observed in one case with Rotor’s type of hyperbilirubinemia (R). In the other three cases of Dubin-Johnson syndrome (DJ), an abnormal disappearance curve with a secondary rise of serum BSP 60 and 120 minutes after intravenous administration, was observed, although the hepatic disappearance rates of both ICG and BSP were normal. A two-compartmental analysis revealed that the depressed hepatic dye clearance in R might be due to defects both in the uptake and in the excretion of dye by the liver, and there seems to be an excretory defect of the dye in DJ.
The studies on the transport maximum (Tm) and the relative storage capacity (S) of ICG demonstrated the reduction of Tm and S in R, while decreased Tm and normal S in DJ. These results suggest that the current diagnostic criteria for DJ and R would be based upon the dye transport distinction in patients with the same basic hyperbilirubinemia.
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Namihisa, T., Yamaguchi, K. & Nambu, M. The constitutional conjugated hyperbilirubinemia. Gastroenterol Jpn 8, 217–223 (1973). https://doi.org/10.1007/BF02779902
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DOI: https://doi.org/10.1007/BF02779902