HealthCentral.com

Crigler-Najjar syndrome



Liver anatomy
Liver anatomy


Crigler-Najjar syndrome

Alternative Names:

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

Phototherapy 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 a pregnancy and have a family history of Crigler-Najjar.

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




Symptoms Checker