Dubin-Johnson syndrome

Definition
Dubin-Johnson syndrome is an inherited disorder characterized by mild jaundice throughout life.

Causes, incidence, and risk factors

Dubin-Johnson syndrome is a very rare autosomal recessive disorder, which means that 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 is dysfunctional in people with this condition. Bilirubrin is a product of the liver’s metabolism of worn-out red blood cells and 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 take on a yellow tinge. Severely high levels 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 that may not appear until puberty or adulthood is the only symptom of Dubin-Johnson syndrome.

Signs and tests

Tests that may be abnormal include:

     
  • Serum bilirubin  
  • Urinary coproporphyrin levels (mostly coproporphyrin I in Dubin-Johnson patients versus coproporphyrin III in normal subjects)  
  • Liver biopsy

Treatment
No specific treatment is required.

Expectations (prognosis)
The prognosis for this condition is very positive. Dubin-Johnson syndrome generally does not shorten the patient’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 progressively worsens.  
  • Abdominal pain or other symptoms are also present (may indicate another disorder is causing the jaundice).

Prevention
Genetic counseling may be valuable for prospective parents with a family history of Dubin-Johnson syndrome.

Johns Hopkins patient information

Last revised: December 4, 2012
by Janet G. Derge, M.D.

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