Dubin-Johnson syndrome

Dubin-Johnson syndrome is a condition characterized by jaundice, which is a yellowing of the skin and whites of the eyes. In most affected people jaundice appears during adolescence or early adulthood. Jaundice is typically the only feature of Dubin-Johnson syndrome, but some people can experience weakness, mild abdominal pain, nausea, or vomiting. In most people with Dubin-Johnson syndrome, certain deposits build up in the liver but do not seem to impair liver function. The deposits make the liver appear black when viewed with medical imaging.

Rarely, jaundice develops soon after birth in individuals with Dubin-Johnson syndrome. Affected infants typically also have enlarged livers (hepatomegaly) and a severely reduced ability to produce and release a digestive fluid called bile (cholestasis). As these children get older, their liver problems go away and they usually do not have any related health problems later in life.

Other names

Black liver-jaundice syndrome; Chronic idiopathic jaundice; Chronic idiopathic jaundice with pigmented liver; DJS; Dubin-Sprinz syndrome; Hyperbilirubinemia II; Hyperbilirubinemia, Dubin-Johnson type; Jaundice, chronic idiopathic

Inheritance pattern

Autosomal recessive

Related genes

ABCC2

Source: MedlinePlus Genetics, U.S. National Library of Medicine.