Dubin-Johnson syndrome

Definition

Dubin-Johnson syndrome is an inherited disorder characterized by 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 transport of bilirubin from the liver does not work correctly in people with this condition. Bilirubin is a product of the liver's break-down of worn-out red blood cells. It is normally sent 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. Severely high levels of bilirubin can damage the brain and other organs.

People with Dubin-Johnson syndrome have life-long low-grade jaundice that may be aggravated by alcohol, pregnancy, oral contraceptive use, infection, and other environmental factors that affect the liver.

Symptoms

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

Exams and Tests

Tests that may be abnormal include:

• Serum bilirubin
• Urinary coproporphyrin levels
• Liver biopsy

Treatment

No specific treatment is required.

Outlook (Prognosis)

The prognosis for this condition is very positive. Dubin-Johnson syndrome generally does not shorten the patient's life span.

Possible Complications

Complications are unusual but may include the following:

• Severe jaundice
• Reduced liver function

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.
• 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.