Dubin-Johnson syndrome

An inherited defect of bile metabolism that causes retention of conjugated bilirubin in hepatic cells. The patient is asymptomatic except for mild intermittent jaundice. No treatment is required.


Dubin-Johnson syndrome is an inherited condition that results from an autosomal recessive genetic trait. The main feature of this disorder is persistent, mild jaundice, leading to yellowing of the skin and the whites of the eyes. However, these symptoms might not be noticeable until the individual reaches puberty or even adulthood. The syndrome originates from a defect in the liver’s transportation system for bilirubin, a primary pigment found in bile. This defect leads to a buildup of bilirubin in the liver.


There is currently no specific cure for this condition. Those afflicted are recommended to abstain from alcohol and steer clear of certain drugs that are metabolized by the liver, such as oral contraceptives. Genetic counseling could be a valuable resource for potential parents with a familial history of Dubin-Johnson syndrome.


 


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