Crigler-Najjar syndrome


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Liver anatomy
Liver anatomy
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.


Support Groups


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.



Review Date: 08/11/2006
Reviewed By: Brian Kirmse, MD, Mount Sinai School of Medicine, Department of Human Genetics, New York, NY. Review provided by VeriMed Healthcare Network.

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