rotor syndrome

Introduction

Syndrome similar to Dubin-Johnson syndrome.

Pathology

• hepatic uptake & conjugation of bilirubin is normal
• excretion of conjugated bilirubin into bile is impaired

Genetics

autosomal dominant

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

• bilirubin metabolism, inborn error