|Reviewed by Carl Kuschel, Simon Chin, and Stephen Mouat (Paediatric Gastroenterology, Starship Hospital)|
|Background||Stool Colour Chart||1st Line Investigations||2nd Line Investigations|
|Other Investigations||Treatment||Related Documents|
Conjugated hyperbilirubinaemia is relatively common occurrence in neonates. Generally, the direct (conjugated) fraction of bilirubin should not be greater than 20mcmol/L, or more than 10% of the total bilirubin if the bilirubin is greater than 200mcmol/L. Most commonly, it is seen in extremely immature infants who are recovering from their immediate neonatal illnesses, and who have had prolonged intravenous nutrition.
The list of possible causes is extensive and a decision to investigate will depend on the clinical circumstances. Investigations should be targeted towards either
Therefore, the form below is a template for investigation - this is not an exhaustive list and other investigations may be warranted. In general, first line investigations should be performed prior to discussing the case with the Paediatric Gastroenterology Service. Other investigations should be performed as indicated.
Referral to the Paediatric Gastroenterology service should be considered early if:
Remember: Identifying one possible cause does not exclude co-existent pathology. For example, it is entirely possible to have both CMV and biliary atresia as dual pathologies.
Stool colour is a useful screen for detecting biliary obstruction (primarily, biliary atresia). It is imperative that stools are examined, rather than a history obtained from parents or other caregivers. Stools in biliary obstruction are persistently pale. Urine colour may be dark or orange.
Adapted from Children’s Liver Disease Foundation. Protocol for community healthcare professionals. Early identification of liver disease in infants. 2002.
|Full Blood Count and film
and conjugated bilirubin
|Liver function tests - specify:
Often low in preterm infants.
If assessing synthetic function, consider a coagulation screen
|INR and/or full coagulation screen|
|Blood group and Coombs|
|Liver ultrasound scan
|Thyroid function tests|
|α1 Antitrypsin phenotype|
(can be obtained from the obstetric record as necessary)
|Maternal toxoplasma serology|
|Maternal Syphilis status|
|Maternal Rubella status|
|Maternal Hepatitis B status|
|Urine sample||bacterial culture|
|Urine organic acids|
|Urine amino acids|
|Serum amino acids|
|Plasma Lactate and
|Herpes simplex PCR
(if clinically suspected)
|Other acquired and congenital infections||Hepatitis A Virus IgM|
|Epstein Barr Virus serology|
(very uncommon cause in the initial perinatal period)
|Urine bile acids
(bile acid synthetic defects)
|Very long chain fatty acids
|White Blood Cell enzymes or
Bone Marrow aspirate
|Transferrin isoelectric focusing (congenital disorders of glycosylation)|
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