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 a chemical called 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 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 the only symptom of Dubin-Johnson syndrome.
Signs and tests
The following tests can help diagnose this syndrome:
The outlook is very positive. Dubin-Johnson syndrome generally does not shorten a person's lifespan.
Complications
Complications are unusual, but may include the following:
Reduced liver function
Severe jaundice
Calling your health care provider
Call your health care provider if any of the following occurs:
Jaundice is severe
Jaundice gets worse over time
You also haveabdominal pain or other symptoms (which may be a sign that 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.
References
Berk P, Korenblat K. Approach to the patient with jaundiceor abnormal liver test results. In Goldman L, Schafer AI, eds. Cecil Medicine. 24th ed. Philadelphia, Pa: Saunders Elsevier; 2011:chap 149.
Review Date:
9/3/2012
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. Health Solutions, Ebix, Inc.
