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Diseases

Genetic and Rare Diseases Information Center (GARD)

Crigler Najjar syndrome, type 2


Other Names for this Disease
  • Crigler-Najjar syndrome, type II
  • Arias syndrome
  • Bilirubin uridinediphosphate glucuronosyltransferase deficiency type 2
  • Bilirubin-UGT deficiency type 2
  • Crigler-Najjar syndrome type 2
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Treatment

Newline Maker

How might Crigler Najjar syndrome, type 2 be treated?

Treatment for Crigler Najjar syndrome, type 2 is based on trying to reduce bilirubin levels. As a result it is commonly treated with aggressive phototherapy and phenobarbitol. For severe disease, calcium gluconate, intravenous fluids, and albumin may be recommended. Severely affected patients have been treated with plasmapheresis and even liver transplantation. These options may be most relevant for individuals with the more severe type I disease. In type II disease, much of the literature supports that long-term reduction in serum bilirubin levels can be achieved with continued administration of phenobarbital.[1][2][3] We recommend that you continue to work closely with your primary health care provider in monitoring your bilirubin levels and the effectiveness of the prescribed therapy.
Last updated: 1/19/2011

References
  1. Crigler Najjar syndrome. MedlinePlus. http://www.nlm.nih.gov/medlineplus/ency/article/001127.htm. Accessed 1/19/2011.
  2. Crigler Najjar syndrome, type II. Online Mendelian Inheritance in Man. http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=606785. Accessed 1/19/2011.
  3. Carey RG, Balistreri WF. Metabolic Diseases of the Liver. In: Kliegman, Nelson . Texbook of Pediatrics, 18th ed. Philidelphia PA: Saunders; 2007;


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Clinical Trials & Research for this Disease

  • Orphanet lists European clinical trials, research studies, and patient registries enrolling people with this condition. 
Other Names for this Disease
  • Crigler-Najjar syndrome, type II
  • Arias syndrome
  • Bilirubin uridinediphosphate glucuronosyltransferase deficiency type 2
  • Bilirubin-UGT deficiency type 2
  • Crigler-Najjar syndrome type 2
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.