Dubin-Johnson syndrome

Last reviewed 01/2018

Dubin-Johnson syndrome is an autosomal recessive disorder characterised by a chronic, benign intermittent jaundice and a predominantly conjugated hyperbilirubinaemia with bilirubinuria. It is most frequent in the Middle East among Iranian Jews.

It results from defective hepatocyte excretion of conjugated bilirubin and other organic anions into bile. Hepatic uptake is usually normal.

Presentation is usually with vague symptoms - anorexia, malaise, some diarrhoea, and jaundice. Pruritus is absent. It may occur during pregnancy or after taking oral contraceptives, both of which reduce hepatic excretory function.

Serum alkaline phosphatase and bile acid levels are normal. Liver biopsy reveals diagnostic pigment granules.

There is no recognised treatment. Prognosis is excellent.