rotor syndrome

Syndrome similar to Dubin-Johnson syndrome. Pathology: 1) hepatic uptake & conjugation of bilirubin is normal 2) excretion of conjugated bilirubin into bile is impaired Genetics: autosomal dominant Clinical manifestations: patients are generally asymptomatic. Laboratory: 1) plasma bilirubin diglucuronide ranges from 2-20 mg/dL 2) urine coproporphyrin levels are normal or slightly increased 3) IV sulfobromophthalein does not result in a 2nd plasma peak Radiology: -> oral cholecystograms do visualize Management: 1) prognosis is excellent 2) phenobarbital may lower serum bilirubin diglucuronide in some patients

Properties

ACCUMULATION: bilirubin diglucuronide

References

Saunders Manual of Medical Practice, Rakel (ed), WB Saunders, Philadelphia, 1996, pg 372