Genetic Liver Diseases

Treatment of Dubin-Johnson Syndrome

Dubin-Johnson sydrome is a rare liver disorder characterized by mild jaundice throughout life. It is caused by an incomplete processing of bilirubin, a product of the liver's metabolism of worn-out red blood cells normally sent into the digestive system. When bilirubin is not properly processed, it builds up in the bloodstream and consequently the skin and the whites of the eyes take on a yellow tinge. It may be aggravated by alcohol, pregnancy, oral contraceptive use, infection and other environmental factors affecting the liver. Severely high levels can damage the brain and other organs.

Diagnosis

Mild jaundice, which may not appear until puberty or adulthood, is the only symptom of Dubin-Johnson syndrome. Diagnostic tests may include bilirubin tests, urine tests or liver biopsies.

Treatment

No treatment is usually required, although in some cases phenobarbital is used to decrease bilirubin. Possible complications which may need treatment include reduced liver function. The prognosis for this condition is very good. It does not generally shorten the patient's life span.