The infant with prolonged jaundice: investigation and management

Abstract 

Jaundice for more than 14 days in the newborn is pathological and requires investigation. Minimum investigations include serum total and conjugated bilirubin. Conjugated hyperbilirubinaemia, dark urine and pale stools are pathognomic of the neonatal hepatitis syndrome which should be investigated urgently. The neonatal hepatitis syndrome has many causes and should be investigated by a structured protocol. The most important differential diagnosis is biliary atresia and affected infants should have a Kasai portoenterostomy carried out by an experienced surgeon, ideally before 60 days of age.

Genetic causes of the neonatal hepatitis syndrome are increasingly recognized and early diagnosis facilitates genetic advice and, in some situations, specific treatment.

The management of cholestasis is largely supportive, consisting of aggressive nutritional support with particular attention to fat-soluble vitamin status. The use of ursodeoxycholic acid is associated with improvement in biochemical measures of cholestasis and may improve the natural history of cholestasis in some circumstances.

Outcome is dependent on aetiology. In idiopatic neonatal hepatitis, more than 90 make a complete biochemical and clinical recovery.

Keywords: neonatal jaundice, biliary atresia, neonatal hepatitis syndrome, Allagile's syndrome, alpha-1 antitrypsin deficiency

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• f1 E-mail: Pat.Mckiernan@bhamchildrens.wmids.nhs.uk