Conjugated Hyperbilirubinaemia in the neonate
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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:
- confirming a clinically suspected diagnosis, or
- excluding a condition for which there is an available treatment.
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:
- there is progression of liver disease or liver failure
- no clear cause is identified
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 Chart
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. Stools that are pale or acholic require urgent investigation.
First Line Investigations
These should be done prior to consulting the Gastroenterology Service.
|Full Blood Count and film|
and conjugated bilirubin
|Liver function tests -
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 Syphilis status
Maternal Rubella status
Maternal Hepatitis B status
Second Line Investigations
Second line investigations that are relatively easy to obtain and may be considered early are:
|Urine organic acids|
|Urine amino acids|
|Serum amino acids|
(if clinically suspected)
These should only be ordered after discussion with a specialist from the Paediatric Gastroenterology service and include:
|Other acquired and
Hepatitis A Virus IgM
Epstein Barr Virus serology
Stool Enterovirus (ECHO, coxsackie)
HCV (very uncommon cause in the initial perinatal period)
|Triglycerides and Cholesterol|
(bile acid synthetic defects)
|Very long chain fatty
|White Blood Cell
enzymes or Bone Marrow aspirate
|Transferrin isoelectric focusing (congenital disorders of glycosylation)|
- If no surgically-correctable lesion is identified, the primary focus is to provide adequate nutrition and vitamin supplementation.
- Malabsorption of long-chain fatty acids and fat-soluble vitamins is common, which may impact on growth, coagulation, and bone mineralisation.
- Consider early referral to a dietitian if they are not already involved.
- Establishing enteral feeds is a priority so that IVN can be discontinued.
Fat-soluble vitamins should be administered until some weeks after resolution of the jaundice. Usual doses are:
- Vitamin K: 2-2.5mg/day (orally)
- Vitadol C: 1ml daily (contains vitamin A 7500iU/ml)
- Vitamin D: 30-50 nanograms/kg once daily
- Micelle E: 0.5ml/day
The gastroenterology service may advise commencing
ursodeoxycholic acid (URSO) at a dose of 20-30mg/kg/day in 2
URSO is a naturally-occurring bile acid that stimulates bile flow.
Specific therapies may be directed at any identifiable underlying cause.
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- Date last published: 31 August 2007
- Document type: Clinical Guideline
- Services responsible: Neonatology
- Owner: Newborn Services Clinical Practice Committee
- Editor: Sarah Bellhouse
- Review frequency: 2 years
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