Abnormal sulfobromophthalein metabolism in Rotor's syndrome and obligate heterozygotes
E Wolpert, FM Pascasio, AW Wolkoff… - New England Journal …, 1977 - Mass Medical Soc
E Wolpert, FM Pascasio, AW Wolkoff, IM Arias
New England Journal of Medicine, 1977•Mass Medical SocRotor'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 …
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 …
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), . . .
The New England Journal Of Medicine