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Glucuronyl transferase deficiency (type I Crigler-Najjar); Arias syndrome (type II Crigler-Najjar)
Light treatment (phototherapy) is needed on a regular basis throughout life. In infants this is done using bilirubin lights (bili or 'blue' lights). Phototherapy becomes less successful after age 4, because thickened skin blocks the light.
Liver transplantation has been used successfully in some people with type 1 disease.
Blood transfusions may help control the amount of bilirubin in blood plasma. Calcium compounds are sometimes used to bind with and remove bilirubin in the gut.
The drug phenobarbitol is sometimes used to treat Arias syndrome (type 2).
Milder forms of the disease (type 2) do not cause severe toxicity, liver damage, or changes in thinking during childhood. People affected still have jaundice, but they have fewer symptoms and less organ damage.
Infants with the severe form of the disease (type 1) may continue to have jaundice into adulthood, and may need daily treatment. If left untreated, this severe form of the disease will lead to death in childhood.
People with this condition who reach adulthood will develop brain damage due to jaundice (kernicterus), even with regular treatment. The life expectancy for type 1 disease is 30 years.
Possible complications include:
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 jaundice that does not go away.
Carey RG, Balistreri WF. Metabolic Diseases of the Liver. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics. 18th ed. Philadelphia, Pa: Saunders Elsevier; 2007: chap 354.
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