Dubin-Johnson syndrome

Definition

Dubin-Johnson syndrome (DJS) is a disorder passed down through families (inherited). In this condition, you may have mild jaundice throughout life.

Causes

DJS 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 syndrome interferes with the body's ability to move bilirubin from the liver. When the liver breaks down worn out red blood cells, it produces bilirubin. It normally moves through the bile produced by the liver and into the bile ducts, past the gallbladder, and into the digestive system.

When bilirubin is not properly processed, it builds up in the bloodstream. This causes the skin and the whites of the eyes to turn yellow. This is called jaundice. Severely high levels of bilirubin can damage the brain and other organs.

People with DJS have lifelong mild jaundice that may be made worse by:

Symptoms

Mild jaundice, which may not appear until puberty or adulthood, is most often the only symptom of DJS.

Exams and Tests

The following tests can help diagnose this syndrome:

Treatment

No specific treatment is required.

Outlook (Prognosis)

The outlook is very positive. DJS generally does not shorten a person's lifespan.

Possible Complications

Complications are unusual, but may include the following:

When to Contact a Medical Professional

Call your health care provider if any of the following occurs:

Prevention

If you have a family history of DJS, genetic counseling may be helpful if you plan to have children.


Review Date: 7/12/2018
Reviewed By: Laura J. Martin, MD, MPH, ABIM Board Certified in Internal Medicine and Hospice and Palliative Medicine, Atlanta, GA. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

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