Conjugated Hyperbilirubinaemia

 

Reviewed by Carl Kuschel, Simon Chin, and Stephen Mouat (Paediatric Gastroenterology, Starship Hospital)
August
2007
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Background Stool Colour Chart 1st Line Investigations 2nd Line Investigations
Other Investigations Treatment   Related Documents

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

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

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.

Healthy
stools

         

 

Suspicious
stools

       

Adapted from Children’s Liver Disease Foundation. Protocol for community healthcare professionals. Early identification of liver disease in infants. 2002.

First Line Investigations

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

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

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

  1. 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.
  2. 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
  3. 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.
  4. Other treatments
    • Specific therapies may be directed at any identifiable underlying cause.



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