Sulfobromophthalein Tolerance Test in Patients with Dubin-Johnson Syndrome and their Relatives

Abstract

The rate of removal of sulfobromophthalein (BSP) sodium from the blood is a function of the secretion into the bile, which is a rate limiting process (transport maximum, or Tm) and the relative storage capacity (S) of the dye in the hepatic parenchyma. By a constant infusion of BSP at different rates, it is possible to measure these two processes. The relative storage capacity and Tm of BSP were both reduced in 13 patients with hepatocellular and obstructive jaundice. In the Dubin-Johnson syndrome, Tm approaches zero, while the relative storage capacity of BSP is entirely normal. This test is therefore pathognomonic for the familial direct hyperbilirubinemia. Constant infusion of BSP in 19 first degree relatives of patients with Dubin-Johnson syndrome and Rotor syndrome failed to identify carriers of the disease. The normal Tm of BSP in the parents of Dubin-Johnson syndrome patients favors an autosomal recessive inheritance.