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Definition:

Dubin-Johnson syndrome is a disorder passed down through families (inherited) in which a person has 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 syndrome interferes with the body's ability to move bilirubin from the liver. Bilirubin is produced when the liver breaks down worn-out red blood cells. 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 and causes the skin and the whites of the eyes to turn yellow (jaundiced). Severely high levels of bilirubin can damage the brain and other organs.

People with Dubin-Johnson syndrome have life-long mild jaundice that may be made worse by:

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


Symptoms:

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



Signs and tests:

The following tests can help diagnose this syndrome:



Treatment:

No specific treatment is required.



Support Groups:



Expectations (prognosis):

The outlook is very positive. Dubin-Johnson syndrome generally does not shorten a person's life span.



Complications:

Complications are unusual but may include the following:

  • Severe jaundice
  • Reduced liver function


Calling your health care provider:

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

  • Jaundice is severe.
  • Jaundice gets worse over time.
  • Abdominal pain or other symptoms are also present (may indicate another disorder is causing the jaundice).


Prevention:

Genetic counseling may be helpful for people who wish to have children and have a family history of Dubin-Johnson syndrome.




Review Date: 7/22/2008
Reviewed By: David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997- A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.
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