Jaundice

Question 1

What are the functions of the liver?

Answer 1

Producing the important proteins
Albumin
Clotting factors

Metabolism of carbs, fats and proteins

Storage for by products like glycogen

Excretes toxic products

Question 2

What is in LFT’s?

Answer 2

Bilirubin
ALT/AST (elevated in hepatitis)
Alkaline phosphate
Gamma glutamyl transferase (GGT) (elevated in biliary disease)

Question 3

What LFT is not used in children?

Answer 3

Alkaline phosphatase as they tend to have higher levels than in adults

Question 4

What are the tests to assess liver function?

Answer 4

Coagulation (prothrombin time)
Albumin
Bilirubin
(Blood glucose)
(Ammonia)

Question 5

What is the most common liver disease in children?

Answer 5

Jaundice

Question 6

What are the signs of jaundice in children?

Answer 6

Encephalopathy
Epistaxis
Spider navi and petechiae
Splenomegaly
Hepatorenal failure
Clubbing
Ascites
Cholestasis = pruritis, pale stools and dark urine

Question 7

At what level of bilirubin is jaundice visible in the sclera?

Answer 7

> 40-50umol/l

Question 8

What is the metabolism of bilirubin?

Answer 8

Erythrocytes break down to Haem then to unconjugated bilirubin

Binds to albumin and transported to the liver. Conjugated to UDP

This is then secreted in bile to small intestine (urobilinogen)

The excreted by kidneys or in stool

Question 9

What are the 3 classifications of jaundice?

Answer 9

Pre-hepatic (uncojugated)
Intrahepatic jaundice - mix of conjugated and unconjugated
Post-hepatic jaundice (cholestasis) - mostly conjugated

Question 10

When is early neonatal jaundice?

Answer 10

<24Hrs old
-Always pathological
Caused by haemolysis and sepsis

Question 11

When is intermediate neonatal jaundice?

Answer 11

24hrs to 2 weeks

Causes = physiological, breast milk, sepsis and haemolysis

Question 12

When is prolonged neonatal jaundice?

Answer 12

> 2weeks

Causes = extrahepatic obstruction, neonatal hepatitis, hypothyroidism and breast milk

Question 13

Why do infants have physiological jaundice?

Answer 13

They have a shorter RBC life span (80-90) days
Immature liver function

Its unconjugated jaundice and develops after first day of life

Question 14

What is breast milk jaundice?

Answer 14

Reasons are unclear

• Inhibition of UDP?
• Increased enterohepatic circulation?

Its unconjugated and can persist for up to 12 weeks

Question 15

What is kernicterus?

Answer 15

Unconjugated bilirubin (fat soluble and water insoluble) = crosses the BBB

=Deposits in brain

Question 16

What are the S+S of kernicterus?

Answer 16

Encephalopathy
Poor feeding, lethargy and seizures

Late = severe choreoathetoid cerebral palsy, learning difficulties and deafness

Question 17

What is the treatment of kernicterus?

Answer 17

Phototherapy = 450nm

Converts bilirubin into a water soluble isomer

Question 18

What are other causes of early/intermediate unconjugated infant jaundice?

Answer 18

Sepsis (Urine, blood and TORCH screen)
Haemolysis (bloods)
Abnormal conjugation (genotype)

Question 19

What are conjugated causes in prolonged (>2w) infant jaundice?

Answer 19

Anatomical (biliary obstruction)

Neonatal hepatitis

Question 20

What are unconjugated causes in prolonged (>2w) infant jaundice?

Answer 20

Hypothyroidism

Breast-milk jaundice

Question 21

What is the most important test in prolonged jaundice?

Answer 21

“split” bilirubin test

Question 22

What are the 3 most common biliary obstruction?

Answer 22

Biliary atresia (conjugated = pale stool) = USS and liver biopsy
Choledoal cyst (conjugated = pale stool) = USS
Alagille syndrome = genotype

Question 23

What is biliary atresia?

Answer 23

Inflammatory of bile ducts leading to obstruction

Present with worsening conjugated juandice = pale stool and dark urine

Progresses to liver failure

Most common indication for transplantation in children

Question 24

What is the treatment for biliary atresia?

Answer 24

Kasai portoenterostomy