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Crigler-Najjar syndrome

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Crigler-Najjar syndrome is a rare form of congenital non-haemolytic hyperbilirubinaemia.

Type I Crigler-Najjar syndrome is characterised by a total deficiency of hepatic glucuronyl transferase. It is inherited as an autosomal recessive. Conjugated bilirubin is absent from the serum. The majority die with kernicterus in the first year of life. Phototherapy can reduce the serum bilirubin by 50% and may be performed at home.

Type II Crigler-Najjar syndrome is characterised by a partial deficiency of glucuronyl transferase. It is inherited as an autosomal dominant. Patients given phenobarbital often survive into adult life. Phototherapy may be used to lower the serum bilirubin level.


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The content herein is provided for informational purposes and does not replace the need to apply professional clinical judgement when diagnosing or treating any medical condition. A licensed medical practitioner should be consulted for diagnosis and treatment of any and all medical conditions.

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