Dubin-Johnson syndrome

• 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:

  • Liver biopsy
  • Serum bilirubin
  • Urinary coproporphyrin levels
• Treatment

  No specific treatment is required.

• 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.