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 General Gastroenterology

Dubin-Johnson syndrome

Dubin-Johnson syndrome

Digestive system organs
Digestive system organs

Definition:

Dubin-Johnson syndrome is an inherited disorder characterized by mild jaundice throughout life.

Causes, incidence, and risk factors:

Dubin-Johnson syndrome is a very rare genetic disorder. In order to inherit the condition, a child must get a copy of the defective gene from both parents.

The transport of bilirubin from the liver does not work correctly in people with this condition. Bilirubin is a product of the liver's break-down of worn-out red blood cells. It is normally sent into the digestive system. When bilirubin is not properly processed, it builds up in the bloodstream and causes the skin and the whites of the eyes to turn yellow. Severely high levels of bilirubin can damage the brain and other organs.

People with Dubin-Johnson syndrome have life-long low-grade jaundice that may be aggravated by alcohol, pregnancy, oral contraceptive use, infection, and other environmental factors that affect the liver.

Symptoms:

Mild jaundice, which may not appear until puberty or adulthood, is the only symptom of Dubin-Johnson syndrome.

Signs and tests:

Tests that may be abnormal include:


Review Date: 10/13/2006
Reviewed By: Jenifer K. Lehrer, MD, Department of Gastroenterology, Frankford-Torresdale Hospital, Jefferson Health System, Philadelphia, PA. Review provided by VeriMed Healthcare Network.

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