요약

• Autosomal-recessive condition characterised by a predominantly conjugated hyperbilirubinaemia.
• Patients are generally asymptomatic and jaundice may be an incidental finding.
• Over 50% of the serum bilirubin is conjugated and bilirubinuria is typically present.
• Symptomatically similar to Dubin-Johnson syndrome but the liver is histologically normal.
• A benign condition with a normal life expectancy, and no specific therapy is required.