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Liver disease

Crigler-najjar syndrome type 1

Crigler-Najjar syndrome type 1 is a genetic condition in which the liver cannot process bilirubin. The condition is usually suspected shortly after birth when the baby fails to clear jaundice. Treatment includes exchange blood transfusion or phototherapy as failure to reduce the bilirubin levels can cause brain damage. The long term treatment for this condition is phototherapy in which the child has to lay under lights for many hours a day. In school age children this may increase to about fifteen hours a day so interfering considerably with schooling and daily life. In these circumstances liver transplantation either whole liver replacement or partial (auxiliary) liver transplantation is the treatment of choice. A mild variant of the condition called type 2 is less serious and jaundice responds to drugs like Phenobarbitone.

Inheritance patterns
Autosomal recessive.

Prenatal diagnosis
Prenatal diagnosis is possible. Analysis of amniotic fluid or chorionic villus sampling (CVS) has been used to establish the diagnosis in the fetus.

View Wilson's disease Wilson's disease  |  Tyrosinaemia type i View Tyrosinaemia type i

Medical written July 2001 by Dr Anil Dhawan. Last updated October 2006 by Professor Anil Dhawan, Consultant Paediatric Hepatologist, Paediatric Liver Centre, King's College Hospital, London, UK.

 

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