Jaundice

Question 1

What LFT is raised in heatocellular damage e.g. hepatitis?

Answer 1

ALT/AST

Question 2

What LFT is raised in biliary diease?

Answer 2

Alkaline phosphatase

Gamma glutamyl transferase (GGT)

Question 3

How does paediatric liver disease present?

Answer 3

Jaundice

Incidental finding

Rarely with signs of chronic liver disease: epistaxis, encephalopathy, ascities, clubbing, hypotonia, spider navei, brusing, growth faliure

Question 4

What is jaundice?

Answer 4

Yellow discolouration of skin and tissues due to accumulation of excess bilirubin. Most obvious in sclera

Usually visiable when total bilirubin >40-50 umol/L

Normal in neontaes 1-13umol/L
Normal in children up to 18 1-8 umol

Question 5

What are the different causes for Jaundice?

Answer 5

Pre-hepatic e.g. increased haemolysis

Intrahepatic- liver diease

Post- hepatic- obstruction stopping bile from getting out e.g. cholestasis

Question 6

What are the causes of neonatel jaundice (neonate=infant less 4 weeks old)?

Answer 6

<24hrs old always patholgical- haemolysis or sepsis

24hrs-2weeks old can be breast mild, sepsis or haemolysis or physiological

> 2 weeks old - extrahepatic obstruction, neonatal hepatitis, hypothryoidism, breast milk

Question 7

Why does physiological jaundice occur?

Answer 7

Shorter life span of RBCs in infants, so more turn over.
Relative polycythaemia
Relative immaturity of liver function (unconjugated jaundice)

Question 8

explain Breast milk Jaundice

Answer 8

We don’t really understand why but babies who are breast fed are more likely to develop jaundice.

Will be unconjugated jaundice and last up to 12 weeks.

Question 9

Why is it so important babies with jaundice are assessd?

Answer 9

Unconjugated bilirubin is fat-soluable/ water insoluable, so it can cross the BBB and deposit in the brain causing brain damage (this condition is called Kernicterus)

Question 10

What is Kernicterus and what are the signs?

Answer 10

Unconjagated bilirubin crosses BBB and depositis in brain which can lead to brain damage.

Early signs: encephalopathy, porr feeding, lethargy, seizures

Consquences: cerebal palsy, learning difficultlies, sensorineural deafness

Question 11

How is jaundice treated to prevent Kernicterus?

Answer 11

For unconjugated jaundice phototherapy 450nm wavelength converts bilirubin in to water soluable isomer.

Or for higher levels:
exchange transfusion, some of babies blood is taken and replaced with bagged blood to reduce bilirubin level.

Question 12

What bilirubin is raised in pre-hepatic jaundice?

Answer 12

Mostly unconjuagted

Question 13

What bilirubin is raised in intrahepatic jaundice?

Answer 13

Mixed unconjugated and conjuagted bilirubin

Question 14

What bilirubin is raised in post-hepatic jaundice?

Answer 14

Mostly conjugated

Question 15

What are the causes of prolonged infant jaundice and how can they be subdivided?

Answer 15

Biliary obstruction (anatomical issue) and Neonatal hepatitis cause mostly conjugated bilirubin rise. (conjuagted jandice is always abnormal)

Where are hypothryoidism and Breast-milk jaundice mostly cause rise in unconjuagted bilirubin.

Question 16

What are the potential causes of biliary obstruction and how do they present?

Answer 16

Biliary atresia and choledochal cyst- both cause conjugated jaundice and pale stools.

algille syndrome- intrahepatic cholestasis, dysmorphism, congential cardiac disease

Question 17

How is biliary atresia treated?

Answer 17

Kasai portoenterostomy (surgery)

Best results if performed before 60 days/9 weeks of life

Question 18

What investigations are done in prolonger jaundice?

Answer 18

Split bilirubin levels (decides in conjugated or unconjuagted bilirubin responsible)

Stool sample

Ultrasound

Liver biopsy

Genotype (if thinking Alagille syndrome)