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    Dubin-Johnson syndrome

    Dubin-Johnson syndrome is a disorder passed down through families (inherited) in which a person has mild jaundice throughout life.

    Causes

    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.

    Exams and Tests

    The following tests can help diagnose this syndrome:

    • Liver biopsy
    • Liver enzyme levels (blood test)
    • Serum bilirubin
    • Urinary coproporphyrin levels

    Treatment

    No specific treatment is required.

    Outlook (Prognosis)

    The outlook is very positive. Dubin-Johnson syndrome 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 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.

    Lidofsky SD. Jaundice. In: Feldman M, Friedman LS,Sleisenger MH, eds. Sleisenger & Fordtran's Gastrointestinal and Liver Disease. 9th ed. Philadelphia, Pa: Saunders Elsevier; 2010:chap 20.

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          Tests for Dubin-Johnson syndrome

            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.

            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. 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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