Stephen J. Schueler, M.D.

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Arias Disease Overview

Another name for Arias Disease is Crigler-Najjar Syndrome.

What is Crigler-Najjar syndrome?
Crigler-Najjar syndrome is a rare inherited disease that can cause jaundice in infants. Crigler-Najjar syndrome follows an autosomal recessive pattern of inheritance. Infants with Crigler-Najjar syndrome have an abnormal gene that fails to make an enzyme (bilirubin glucuronyl transferase) that is needed to help metabolize bilirubin. This causes bilirubin to buildup in the bloodstream to dangerous levels. Crigler-Najjar syndrome is an extremely rare disorder that affects less than 1 in one million births.

What are the symptoms of Crigler-Najjar syndrome?
Symptoms of Crigler-Najjar syndrome include neonatal jaundice, infant fussiness, and poor weight gain.

How does the doctor treat Crigler-Najjar syndrome?
Treatment of Crigler-Najjar syndrome may include phototherapy to lower bilirubin in the bloodstream. Other measures include phenobarbital and liver transplantation.

Continue to Arias Disease Symptoms

Last Updated: Feb 14, 2011 References
Authors: Stephen J. Schueler, MD; John H. Beckett, MD; D. Scott Gettings, MD
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PubMed Arias Disease References
  1. Dennery PA. Pharmacological interventions for the treatment of neonatal jaundice. Semin Neonatol. 2002 Apr;7(2):111-9. [12208095]
  2. Faber BM, Mills JF. Early intravenous nutrition for the prevention of neonatal jaundice. Cochrane Database Syst Rev. 2003;(3):CD003846. [12917992]
  3. Lake JR. Hepatocyte transplantation. N Engl J Med. 1998 May 14;338(20):1463-5. [9580657]
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