Paediatric Protocol
Gastrointestinal: 11.3
January 2001
Prolonged Neonatal Jaundice Protocol
As jaundice is present in up to 90% of newborn babies, liver disease may be difficult
to detect. In practice, if jaundice persists beyond two weeks after birth it should be
investigated even in breast-fed babies.
In a well child, the first step is to examine the stools and urine. If the stools are pale
and the urine contains bilirubin, the child needs urgent referral to a Paediatric
Gastroenterologist to exclude biliary atresia etc.
The second step is to confirm whether the prolonged jaundice is due to an elevation of
conjugated or unconjugated bilirubin in the plasma (split bilirubin).
See Summary Flow chart
Unconjugated Hyperbilirubinaemia
If the estimation of bilirubin indicates that > 85% is unconjugated, then breast milk
jaundice or another cause of unconjugated hyperbilirubinaemia is likely (Table 1).
Appropriate further investigations should include:
1.
2.
3.
4.
5.
H
a
e
m
o
l
y
s
i
s
Full blood count, including reticulocytes
Examination of blood film
Thyroid function tests
Gal-1-Put – i.e. red cell galactose-1-phosphate uridyl transferase
Urine culture
Table 1.
Causes of unconjugated hyperbilirubinaemia
Breast Milk Jaundice
Haemolysis
Blood group incompatibilities (ABO & Rhesus)
Polycythaemia
Extravasated Blood
Increased Enterohepatic Circulation
Pyloric stenosis
Bowel obstruction
Endocrine/Metabolic (also cause conjugated hyperbilirubinaemia)
Hypothyroidism
Hypopituitarism
Hypoadrenalism
Galactosaemia
Sepsis
January 2001
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Dr David Bond
Conjugated Hyperbilirubinaemia
If > 15% of the total bilirubin is conjugated or bilirubin is detected in the urine, liver
disease must be excluded. The differential diagnosis is extensive (Table 2), but it is
essential to exclude biliary atresia early by examining the stools and urine. If the stool
is pigmented, coagulation needs checking, and further investigations should be coordinated by a Paediatric Gastroenterologist looking for causes of neonatal hepatitis.
The key is to arrange a HIDA scan, which should only be performed after five days of
oral Phenobarbitone (5mg/kg/day). This gives sufficient time for the following tests to
be performed:
1. Liver function tests
2. Coagulation screen
3. Blood glucose
4. Full blood count
5. TORCH screen / Hepatitis serology
6. Alpha-1-Antitrypsin level and phenotype (Li. Hep.) or genotype (EDTA)
7. Gal-1-Put
8. Thyroid Function Tests
9. Random Cortisol
10. Plasma amino acids
11. Serum iron and ferritin
12. Urine culture
13. Urine metabolic screen
Immediate referral to a Paediatric Gastroenterologist is mandatory if a neonate has
two or more of the following:






Conjugated hyperbilirubinaemia > 40 mol/l
Pale stools
Bilirubin in urine
Bleeding tendency
Failure to thrive
Recurrent hypoglycaemia
More specialized investigations will include:
1. HIDA Scan after enzyme induction for five days with Phenobarbitone
(5mg/kg/day in a single dose)
2. Abdominal Ultrasound
3. CxR (butterfly vertebrae)
4. Eye examination (posterior embryotoxin)
5. Liver biopsy (after correction of coagulopathy)
6. Echocardiography (pulmonary stenosis)
7. Sweat test
January 2001
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Dr David Bond
 Contact Dr Charlton at Queen’s Medical Centre (Secretary on 42439 or Page via
switchboard). In Dr Charlton’s absence, infants should be referred to the Paediatric
Liver Unit at St.James’ Hospital, Leeds.
Table 2.
Causes of conjugated hyperbilirubinaemia
A. Extrahepatic biliary duct disorders
Biliary atresia
Choledochal cyst
Gallstone
B. Bile Duct Hypoplasia Syndromes (Alagilles)
C. Neonatal Hepatitis Syndrome
Idiopathic Neonatal Hepatitis Syndrome
Intrauterine Infections
Metabolic
Hypothyroidism
Hypopituitarism
-1 antitrypsin deficiency
Galactosaemia
Tyrosinaemia Type 1
Hereditary fructose intolerance
Niemann Pick Type C
Zellweger’s
Cystic fibrosis
Sepsis
Urinary Tract infection
Sepsis
Urinary tract infection
Prolonged parenteral nutrition
January 2001
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Dr David Bond
Flow Chart For Management of Prolonged Neonatal Jaundice
Jaundiced infant
> 2 weeks old
Stools pale
and
NO
bilirubinuria
YES
Initial Investigations:
LFT with split bilirubin
FBC + Reticulocytes
TFT
Gal-1-Put
Urine culture
Urgent referral
to
Paediatric
Gastroenterologist
Unconjugated
bilirubin
Conjugated
bilirubin
> 85 % of total
> 15 % of total
Registrar to chase
results and ensure
formal GP letter written
References
1. Ives N K. Neonatal Jaundice. Current Paediatrics (1997) 7, 67-72.
2. Kelly D A. Hepatology in Investigations in Paediatrics. Addy and Douglas
(eds.) (1994) 123-130. WB Saunders.
3. Roberts, E A. The Jaundiced baby in Diseases of the Liver and Biliary
System in Children. Kelly (ed) (1999) 11-45. Blackwell Science.
January 2001
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Dr David Bond