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Crigler-Najjar syndrome - Treatment

Alternative Names

Glucuronyl transferase deficiency (type I Crigler-Najjar); Arias syndrome (type II Crigler-Najjar)

Treatment:

Phototherapy (light treatment) is needed on an ongoing basis throughout life. In infants this is done using bilirubin lights (bili or 'blue' lights). Phototherapy becomes less successful after 4 years because thickened skin blocks the light.

Liver transplantation has been used successfully for some people with type I disease.

Expectations (prognosis):

Affected individuals who reach adulthood will develop kernicterus despite ongoing treatment. The life expectancy for type I disease has been extended from death in childhood to people now living to at least age 30.

Complications:

Possible complications include:

  • Kernicterus
  • Chronic yellow skin/eyes

Calling your health care provider:

Seek genetic counseling if you are planning to have children and have a family history of Crigler-Najjar.

Call your health care provider if you or your newborn infant has prolonged jaundice.

  • Reviewed last on: 8/11/2006
  • Brian Kirmse, MD, Mount Sinai School of Medicine, Department of Human Genetics, New York, NY. Review provided by VeriMed Healthcare Network.