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.
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