Liver

Question 1

RECAP- functions of the liver?

Answer 1

Produces bile
Produces clotting factors
Role in absorption of fats, proteins and carbs
Glycogen storage
Albumin store
Excretion of toxic products

Question 2

RECAP- which blood tests are included in LFTs?

Answer 2

Bilirubin- can be split into conjugated and unconjugated
ALT/AST
ALP
GGT

Question 3

In which types of liver disease can ALT/AST be elevated?

Answer 3

Hepatocellular damage e.g. hepatitis

Question 4

In which types of liver disease can ALP and GGT be elevated?

Answer 4

Biliary disease

Question 5

LFTs are a better indication of how badly damaged the liver is.

Which tests are better tests to assess the function?

Answer 5

Coagulation- PT, APTT
Albumin
Bilirubin

Question 6

Jaundice is the commonest presentation of liver disease in children. What is jaundice?

Answer 6

Yellow discolouration of the skin and tissues due to accumulation of bilirubin

Question 7

Where is jaundice usually the most noticable?

Answer 7

Sclera of eyes

Question 8

At what levels of bilirubin does jaundice then become more visibly noticed?

Answer 8

When total bilirubin >40-50umol/l

Question 9

Looking for yellowing of the sclera is the best way to differentiate true jaundice and which condition?

Answer 9

Beta-carotene anaemia

->this is what Mr Page had when he ate too many carrots

Question 10

Which cells are broken down to produce bilirubin?

Answer 10

RBCs

Question 11

What is unconjugated bilirubin bound to?

Answer 11

Albumin

->this then gets transported to the liver

Question 12

Which type of bilirubin is water soluble?

Answer 12

Conjugated bilirubin

->this is why unconjugated bilirubin is bound to albumin

Question 13

Summarise the bilirubin metabolism pathway

Answer 13

RBCs -> unconjugated bilirubin (bound to albumin) -> liver to become conjugated

Then this goes into bile

Question 14

So… if there is a pre-hepatic jaundice, where does the porblem occur?

Answer 14

Anywhere in the pathway before the liver, so involves unconjugated bilirubin

Question 15

In intra-hepatic jaundice, where is the issue?

Answer 15

Liver

->both conjugated and unconjugated bilirubin as liver has issues with both

Question 16

What issue is usually the cause of post-hepatic jaundice?

Answer 16

Obstructive cause preventing conjugated bilirubin getting out of the liver

Question 17

Early onset jaundice occurs within 24hrs of birth and is ALWAYS pathological.

What are some of the causes?

Answer 17

Haemolysis, sepsis

Question 18

Intermediate jaundice can occur between 24hrs-2wks after birth.

What are some of the causes?

Answer 18

Physiological
Breast milk
Haemolysis
Sepsis

Question 19

Prolonged jaundice can occur in babies >2wks after birth.

What are some of the causes?

Answer 19

Extrahepatic obstruction
Neonatal hepatitis
Hypothyroidism
Breast milk

Question 20

What type of jaundice is breast milk jaundice?

Answer 20

Unconjugated jaundice

Question 21

Although neonatal jaundice is very common, and often doesn’t require treatment, there is one condition associated with jaundice which is severe.
What is it?

Answer 21

Kernicterus

Question 22

Which type of bilirubin is there in kernicterus?

Answer 22

Unconjugated

Question 23

Why is Kernicterus a potentially severe condition?

Answer 23

The unconjugated bilirubin is fat-soluble, and water insoluble, and can cross the blood-brain barrier.
It is neurotoxic and can deposit in the brain

Question 24

What are the early signs of knericterus?

Answer 24

Encephalopathy
Poor feeding
Lethargy
Seizures

Question 25

What are some of the late consequences of Kernicterus if it's not treated?

Answer 25

Severe choreathetoid cerebral palsy Learning difficulties Sensorineural deafness

Question 26

What treatment is done for unconjugated jaundice to avoid Kernicterus developing?

Answer 26

Phototherapy

Question 27

How common and severe is Gilbert's syndrome?

Answer 27

Common, mild severity

Question 28

How common and severe is Crigler-Najjar syndrome?

Answer 28

V rare, severe

Question 29

Which tests are done to confirm diagnosis of neonatal sepsis?

Answer 29

Urine, blood cultures, TORCH screen

Question 30

Which tests are done to confirm diagnosis of Gilbert's/Crigler-Najjar syndrome?

Answer 30

Genotype/phenotype testing

Question 31

What is prolonged infant jaundice?

Answer 31

Jaundice persisting beyond 2wks of life ->beyond 3wks in preterm infants

Question 32

What are some of the causes of prolonged infant jaundice?

Answer 32

Biliary obstruction Neonatal hepatitis Hypothyroidism ->could be breast-milk or physiological jaundice but more unlikely if more than 2wks

Question 33

Causes of prolonged infant jaundice can be either conjugated or unconjugated. What are the conjugated causes?

Answer 33

Biliary obstruction Neonatal hepatitis ->makes sense as both occur within or after liver

Question 34

Causes of prolonged infant jaundice can be either conjugated or unconjugated. What are the unconjugated causes?

Answer 34

Hypothyroidism Breast-milk jaundice

Question 35

What does conjugated jaundice in infants always require?

Answer 35

Further investigation as always abnormal. The most important test in prolonged jaundice is a 'split' bilirubin to determine the type

Question 36

What are some of the things that can cause biliary obstruction and lead to prolonged jaundice?

Answer 36

Biliary atresia Choledochal cyst Alagille syndrome

Question 37

Symptoms of biliary atresia and choledochal cysts?

Answer 37

Conjugated jaundice Pale stools ->stools are pale because bilirubin is not getting excreted due to the blockage

Question 38

Therefore, if you have a child with prolonged jaundice, what do you always want to check, as well as the type of bilirubin conjugation?

Answer 38

Stool colour

Question 39

What happens in biliary atresia?

Answer 39

Congenital fibro-inflammatory disease of the bile ducts

Question 40

If biliary atresia is not treated, what can it progress to?

Answer 40

Liver failure ->biliary atresia is the most common indication for liver transplant in children

Question 41

Treatment for biliary atresia?

Answer 41

Kasai portoenterostomy ->success rate diminishes rapidly with age, best results if performed before 60 days

Question 42

Which imaging modality is best for assessing the liver for biliary atresia/choledochal cysts?

Answer 42

Ultrasound