Dubin-Johnson syndrome

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 PD, Korenblat KM. Approach to the patient with jaundice or abnormal liver test results. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier;2007:chap 150.

Update Date: 8/14/2010

Updated 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.