rotor syndrome
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Introduction
Syndrome similar to Dubin-Johnson syndrome.
Pathology
- hepatic uptake & conjugation of bilirubin is normal
- excretion of conjugated bilirubin into bile is impaired
Genetics
Clinical manifestations
patients are generally asymptomatic
Laboratory
- plasma bilirubin diglucuronide ranges from 2-20 mg/dL
- urine coproporphyrin levels are normal or slightly increased
- IV sulfobromophthalein does not result in a 2nd plasma peak
Radiology
- oral cholecystograms do visualize
Management
- prognosis is excellent
- phenobarbital may lower serum bilirubin diglucuronide in some patients
More general terms
References
- ↑ Saunders Manual of Medical Practice, Rakel (ed), WB Saunders, Philadelphia, 1996, pg 372