Abnormal Sulfobromophthalein Metabolism in Rotor's Syndrome and Obligate Heterozygotes

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Abstract
Rotor's syndrome is an inheritable disorder characterized by chronic nonhemolytic, predominantly conjugated hyperbilirubinemia without abnormal hepatic pigmentation.1 2 3 4 Because of clinical similarities, Rotor's syndrome has been considered to be a variant of the Dubin-Johnson syndrome.2 , 3 , 5 Recent studies of urinary coproporphyrin excretion, however, demonstrate that the two syndromes are distinct pathophysiologic entities.6 Studies of hepatic transport of sulfobromophthalein in patients with the Dubin-Johnson syndrome reveal a rise in plasma sulfobromophthalein concentration 90 to 120 minutes after intravenous injection of 5 mg of dye per kilogram.7 8 9 10 Moreover, hepatic transport maximum (Tm) is virtually zero, with a normal relative storage capacity (S), . . .