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Dubin-Johnson syndrome

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:

  • Alcohol
  • Birth control pills
  • Environmental factors that affect the liver
  • Infection
  • Pregnancy

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:

  • Reduced liver function
  • Severe jaundice

When to Contact a Medical Professional

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

  • Jaundice is severe
  • Jaundice gets worse over time
  • You also have abdominal pain or other symptoms (which may be a sign that another disorder is causing the jaundice)

Prevention

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

References

Berk PD, Korenblat KM. Approach to the patient with jaundice or abnormal liver tests. In: Goldman L, Schafer AI, eds. Goldman-Cecil Medicine. 25th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 147.

Lidofsky SD. Jaundice. In: Feldman M, Friedman LS, Brandt LJ, eds. Sleisenger and Fordtran's Gastrointestinal and Liver Disease. 10th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 21.

Review Date 8/22/2016

Updated 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, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.

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