21. Liver Problems

Question 1

What is the function of the liver?

Answer 1

Filtration
Clotting factors
Enzyme activation
Bile production
Storage of glycogen, vitamins and minerals

Question 2

What are the liver damage tests?

Answer 2

Bilirubin (conjugated and unconjugated)
ALT/AST- alanine aminotransferase/aspartate aminotransferase- elevated in hepatocellular damage
Alkaline phosphate — elevated in biliary disease (not used in paeds)
Gamma glutmya transferase (GGT)- elevated in biliary Test

Question 3

How do you measure liver function?

Answer 3

Coagulation- prothrombin time/ (PT/INR)

Albumin- chronic liver disease

Bilirubin

Blood glucose

Ammonia

Question 4

What is the most common manifestation of paediatric liver disease

Answer 4

Jaundice- very very common

Incidental finding of abnormal blood test

Question 5

What are the signs of chronic liver disease in children?

Answer 5

Growth failure
Encephalopathy 
Portal hypertension
Spider naevi
Ascites
Clubbing 
Rickets

Question 6

How high is bilirubin to cause jaundice?

Answer 6

> 40-50umol/L

Question 7

What is beta-karetaenemia?

Answer 7

Or changing of the skin due to lots of vegetables. It will only change skin pigment and not the sclera

Question 8

Explain the process of unconjugated bilirubin being formed?

Answer 8

Post mature erythrocytes are broken down all over the body into haem. Haem is broken down into biliverdin which is transmitted into reductase

Question 9

How is conjugated bilirubin excreted?

Answer 9

Unconjugated bilirubin binds to albumin and is transported to the liver. In the liver it is turned into conjugated bilirubin (water soluble)

It then is put into the bile, excreted in the small intestine and excreted in pee or poor

Question 10

What are the different types of jaundice?

Answer 10

Pre hepatic jaundice- problem with haemolysis, increase in unconjugated bilirubin

Hepatic jaundice- problems with the liver e.g. hepatitis. Mix of conjugated and unconjugated

Post hepatic jaundice- problems with the biliary tree, increase in conjugated bilirubin

Question 11

What are the types of neonatal jaundice?

Answer 11

Early (<24 hours) always pathological, caused by haemolysis and sepsis

Intermediate (24hrs-2 weeks) Caused by physiology, breast milk, sepsis, heamolysis

Prolonged (>2 weeks) Caused by obstruction, hepatitis, hypothyroidism, breast milk

Question 12

Why do infants become physiologically jaundiced?

Answer 12

Shorter RBC life span in infants
Relative polycythaemia (high RBC)
Relative immature liver function

Unconjugated jaundice that occurs after the first day of life

Question 13

What is kernocterus?

What are the signs?

What are the complications?

Answer 13

Disease of the brain where unconjugated bilirubin crosses the brain barrier and deposits in the brain

Early signs are encephalopathy, poor feeding, lethargy and seizures

Late consequences include cerebral palsy, learning difficulties and deafness

Question 14

How do you treat kernocterus

Answer 14

Phototherapy- blue light converts bilirubin to water soluble isomer

(Not UV LIGHT)

Question 15

What are some other causes of early jaundice?

Be specific

Answer 15

Sepsis
ABO incompatibility
Rhesus disease
Bruising
G6DP, spherocytosis

Question 16

How do you investigate early jaundice?

Answer 16

Urine and blood glucose
Coombs Test
GD6P assay
Genotype/phenotype (gilberts)

Question 17

What are the different causes of older neonatal jaundice

Answer 17

Anatomical (biliary obstruction)- conjugated
Neonatal hepatitis- conjugated
Hypothyroidism- unconjugated
Breast milk jaundice- unconjugated

Question 18

What is the key message from this lecture?

Answer 18

Conjugated jaundice in children is always abnormal

Question 19

What is another key message from this lecture?

Answer 19

Always assess stool colour I’m infants with prolonged jaundice

Question 20

What are the different types of prolonged jaundice (obstructive)

How do Test for it

Answer 20

Biliary atresia- conjugated jaundice pale stools
Investigate With bilirubin USS, biopsy

Choledochal cyst conjugated jaundice, pale stools
Investigate with bilirubin, USS

Alagille sydrome Intrahepatc cholestasis, dysmorphism, congenital cardiac disease. Investigate using genotpying

Question 21

How does biliary atresia present?

Answer 21

Inflammatory process of bile ducts leading to the destruction of the bile ducts
Prolonged conjugated jaundice
Pale stools, dark urine
Progression to liver failure if not identified and treated

Question 22

What is the treatment of biliary atresia?

Answer 22

Kasai portentersostomy- needs to be done before 60 days
Early diagnosis is key
If not need liver transplant
Doesn’t always cure, can lead to liver transplant in childhood/ Adult life

Question 23

Why do you assess children with jaundice early

Answer 23

Prove it’s not biliary atresia as needs treated ASAP

Question 24

What are some rare causes of prolonged jaundice?

Answer 24

Alpha 1 antitrypsin level
Galactosaemia
Tyrosineamka
Urea cycle defects
Haemochromotosis

Question 25

What are the three things we must remember!

Answer 25

Always ask about stool colour

Test split bilirubin

Conjugated jaundice ALWAYS needs further investigation. Test for biliary atresia until it’s not