Jaundice At > 2 Weeks Of Age

Question 1

What unconjugated causes are there?

Answer 1

Physiological or breast milk jaundice
Infection - particularly UTI
Hypothyroidism 
Haemolytic anaemia e.g G6PD deficiency 
High GI obstruction e.g pyloric stenosis

Question 2

What conjugated causes are there?

Answer 2

Bile duct obstruction

Neonatal hepatitis

Question 3

Jaundice at > 2 weeks (3 weeks if preterm) is called..

Answer 3

Persistent or prolonged jaundice

Question 4

What is important to diagnose promptly ?

Answer 4

Biliary atresia

Question 5

In most cases with persistent jaundice, is the bilirubin conjugated?

Answer 5

Unconjugated- needs to be confirmed on laboratory testing

Question 6

What is the most common cause of prolonged unconjugated hyperbilirubinaemia?

Answer 6

Breast milk jaundice - affects 15 % of healthy breastfed infants (gradually fades by 4-5 weeks of age)

Question 7

Can congenital hypothyroidism cause prolonged jaundice before clinical features of coarse facies, dry skin, hypotonia, constipation etc develop?

Answer 7

Yes

Affected infants should be identified on routine neonatal biochemical screening (Guthrie test)

Question 8

Why is breastfeeding a known cause of jaundice?

Answer 8

In the early days of breastfeeding, milk production is steadily increasing - there may initially be an inadequate supply of milk. Reduced intake can cause less bilirubin to be secreted into the gut and so less is removed from the body, which can be detected as jaundice.

Question 9

Why is it important to ask about feeding in the history?

Answer 9

Breastfeeding = known cause of jaundice

Poor feeding with jaundice can be a symptom of kernicterus- needs prompt recognition and treatment

Question 10

What are the clinical features of kernicterus?

Answer 10

Lethargy
Poor feeding
Irritability, shrill cry
Hypertonia
Opisthotonus (arching of neck and spine due to muscle spasm) 
Seizure

Question 11

Is persistently coloured urine in a baby abnormal?

Answer 11

Yes - a baby’s urine should be colourless

Persistently yellow urine which stains the nappy may indicate liver disease

Question 12

Are pale or chalky stools in a baby abnormal?

Answer 12

Yes

Question 13

Why should you check the child’s newborn blood spot screening programme results?

Answer 13

Because hypothyroidism and CF are possible causes of prolonged jaundice in a neonate - both of these are screened for in the new born spot programme

Question 14

When is the new born blood spot screening programme done and what is it for?

Answer 14

Day 5 of life
Screens for 9 rare but serious conditions:
Sickle cell disease
CF
Congenital hypothyroidism
Inherited metabolic diseases (PKU, MCADD, MSUD, IVA, GA1, HCU)

Question 15

What possible causes are there for prolonged jaundice (extended list) ?

Answer 15

Structural - biliary atresia, choledochal cyst, Alagille’s syndrome
Infective - CMV, EBV, HepB, bacterial, toxoplasmosis
Metabolic - galactosaemia, alpha1 antitrypsin deficiency, storage disease
Genetic - CF, trisomy 21, trisomy 18
Neoplastic - hepatoblastoma, neuroblastoma
Toxic - drug induced
Endocrine - hypothyroidism, panhypopituitarism
Vascular - Budd-Chiari, CCF

Question 16

What investigations should be done?

Answer 16

Urine dip
FBC - raised neutrophils or evidence of anaemia
Coagulation study - check liver’s synthetic function
TFTs - rule out hypothyroidism as cause
Liver panel (AST, ALT, ALP, GGT, total bilirubin, split bilirubin)
Blood culture - preform early to rule out infection in the blood
Galactosaemia screen - important to rule out as easily reversible with a change in diet
TORCH screen

Question 17

What does a split bilirubin tell you?

Answer 17

Ratio of unconjugated bilirubin to conjugated
- a high conjugated bilirubin indicates the liver is working but that it is unable to move the bilirubin out of the liver and into the gut

Question 18

How can galactosaemia present?

Answer 18

Jaundice and hepatomegaly in neonate

Question 19

If conjugated bilirubin is more than 20% of the bilirubin, what does this suggest?

Answer 19

A problem with getting the bilirubin out of the liver - suggests liver disease

Question 20

Will phototherapy breakdown conjugated bilirubin?

Answer 20

No