Abnormal sulfobromophthalein metabolism in rotor's syndrome and obligate heterozygotes

E. Wolpert, F. M. Pascasio, Allan W. Wolkoff, I. M. Arias

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Abstract

Rotor's syndrome is an inheritable disorder characterized by chronic nonhemolytic, predominantly conjugated hyperbilirubinemia without abnormal hepatic pigmentation. Because of clinical similarities, Rotor's syndrome has been considered to be a variant of the Dubin Johnson syndrome. Recent studies of urinary coproporphyrin excretion, however, demonstrate that the two syndromes are distinct pathophysiologic entities. Studies of hepatic transport of sulfobromophthalein in patients with the Dubin Johnson syndrome reveal in a rise in plasma sulfobromophthalein concentration 90 to 120 minutes after intravenous injection of 5 mg of dye per kilogram. Moreover, hepatic transport maximum (Tm) is virtually zero, with a normal relative storage capacity (S), and presumed heterozygotes have either normal sulfobromophthalein metabolism or mildly reduced Tm with normal S. In the present study, plasma clearance and transport of sulfobromophthalein were studied in patients with Rotor's syndrome, phenotypically normal parents, children and siblings and normal controls.

Original languageEnglish (US)
Pages (from-to)1099-1101
Number of pages3
JournalNew England Journal of Medicine
Volume296
Issue number19
StatePublished - 1977

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Hereditary Hyperbilirubinemia
Sulfobromophthalein
Heterozygote
Chronic Idiopathic Jaundice
Liver
Coproporphyrins
Hyperbilirubinemia
Pigmentation
Intravenous Injections
Siblings
Coloring Agents
Parents

ASJC Scopus subject areas

  • Medicine(all)

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Abnormal sulfobromophthalein metabolism in rotor's syndrome and obligate heterozygotes. / Wolpert, E.; Pascasio, F. M.; Wolkoff, Allan W.; Arias, I. M.

In: New England Journal of Medicine, Vol. 296, No. 19, 1977, p. 1099-1101.

Research output: Contribution to journalArticle

Wolpert, E. ; Pascasio, F. M. ; Wolkoff, Allan W. ; Arias, I. M. / Abnormal sulfobromophthalein metabolism in rotor's syndrome and obligate heterozygotes. In: New England Journal of Medicine. 1977 ; Vol. 296, No. 19. pp. 1099-1101.
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