Crigler-Najjar Syndrome | Types of Crigler – Najjar Syndrome | Crigler Najjar Syndrome Type

Crigler-najjar syndrome:

Crigler-najjar syndrome is a type of congenital hyper-bilirubinaemia.

In this, there is congenital absence of the enzyme UDP glucoronyl transferase.

Two types of Crigler-najjar syndrome are described-

Type-I Crigler-najjar syndrome :

It is characterized by the onset of jaundice at birth.

In this, bile secreted is colorless due to the lack of enzyme bilirubin glucoronide.

Patient does not respond to phenobarbitone therapy and kernicterus (a condition with severe neural symptoms, associated with high levels of bilirubin in the blood) usually supervenes.

In those patients, liver transplant is considered as option for treatment.

Type-II Crigler-najjar syndrome:

This variety of Crigler-najjar syndrome is milder form of disease.

In this the onset of jaundice may be delayed till the age of 10yrs.

Bilirubin level id reduced with daily phenobarbitone therapy in type-II.

Type-I is inherited as an autosomal recessive trait while type-II is inherited as an autosomal dominant trait.

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