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Conjugated Hyperbilirubinaemia in the neonate

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Background

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:

  1. there is progression of liver disease or liver failure 
  2. 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

See colour chart bookmark from the Children's Liver Disease Foundation

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.

Test Date Taken Result
Full Blood Count and film    
Total
and conjugated bilirubin
   
Liver function tests - specify:
AST
ALT
GGT
ALP 
   
Blood gas    
Albumin
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     
Ferritin    
Thyroid function tests     
α1 Antitrypsin phenotype     
Urine CMV     
Maternal/congenital infection
(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
reducing substances 
 

Second Line Investigations

Second line investigations that are relatively easy to obtain and may be considered early are:

Test Date Taken Result
Urine organic acids    
Urine amino acids    
Serum amino acids    
Plasma ammonia    
Plasma Lactate and
Pyruvate
   
Herpes simplex PCR
(if clinically suspected)
   

Other Investigations

These should only be ordered after discussion with a specialist from the Paediatric Gastroenterology service and include:

Test Date Taken Result
Other acquired and congenital infections:
Hepatitis A Virus IgM
Adenovirus serology
Epstein Barr Virus serology
Stool Enterovirus  (ECHO, coxsackie)
Parvovirus PCR
HHV6 PCR
HCV  (very uncommon cause in the initial perinatal period)
HIV
   
Triglycerides and  Cholesterol    
Carnitine    
Urine bile acids
(bile acid synthetic defects)
   
Very long chain fatty acids
(peroxisomal disorders)
   
White Blood Cell enzymes or  Bone Marrow aspirate
(storage disorders)
   
Karyotype    
HIDA scan     
Transferrin isoelectric focusing (congenital disorders of glycosylation)    

Treatment

Nutritional support

  • 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.

Vitamins

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

Ursodeoxycholic acid

The gastroenterology service may advise commencing ursodeoxycholic acid (URSO) at a dose of 20-30mg/kg/day in 2 divided doses.
URSO is a naturally-occurring bile acid that stimulates bile flow.

Other treatments

Specific therapies may be directed at any identifiable underlying cause.

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Document Control

  • 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