Dubin-Johnson syndrome

An inherited disorder characterized by mild jaundice throughout life.

Causes, incidence, and risk factors

Dubin-Johnson syndrome is inherited as an autosomal recessive disorder. The transport of bilirubin from the liver into the biliary system is abnormal and the bilirubin accumulates in the liver. Affected people have life-long low-grade jaundice which may be aggravated by alcohol, pregnancy , infection, and other environmental factors.

Signs and tests

Tests which may be abnormal include:

  • chem-20
  • to check
  • bilirubin levels
  • blood chemistry
  • (may show sulfobromophthalein retention)
  • Liver biopsy
  • Treatment

    No specific treatment is available.

    Expectations (prognosis)

    Dubin-Johnson syndrome is compatible with a normal life span.

    Complications

    Complications are unusual but may include:

  • severe
  • jaundice
  • reduced liver function
  • Calling your health care provider

    Call your health care provider if:

  • jaundice
  • is severe
  • jaundice progressively worsens
  • abdominal pain
  • or other symptoms are also present (there may be another disorder causing the jaundice)
  • Prevention

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

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