Liver

Question 1

What are examples of LFTs?

Answer 1

Bilirubin
ALT/AST
Alkaline phosphatase
Gamma glutamyl transferase

Question 2

What causes elevated ALT/AST?

Answer 2

Hepatitis

Question 3

What causes elevated ALP and GGT?

Answer 3

Biliary disease

Question 4

What tests can be used to assess liver function?

Answer 4

Coagulation - prothrombin time, INR, APTT
Albumin
Bilirubin

Question 5

What is the most common manifestation of liver disease in children?

Answer 5

Jaundice

Question 6

Where is jaundice most obvious?

Answer 6

Sclera

Question 7

What level of bilirubin causes visible jaundice?

Answer 7

> 40-50umol/l

Question 8

How is bilirubin metabolised?

Answer 8

Post mature erythrocytes are broke down releasing unconjugated bilirubin
Bilirubin transported to liver via albumin and conjugated
Conjugated bilirubin is stuck into bile

Question 9

What are causes of jaundice in the first 24 hours of life?

Answer 9

Always pathological - haemolysis, sepsis

Question 10

What are causes of jaundice from 24 hours of age to 2 weeks of age?

Answer 10

Physiological
Breast milk
Sepsis
Haemolysis

Question 11

What are causes of jaundice lasting over 2 weeks?

Answer 11

Extrahepatic obstruction
Neonatal hepatitis
Hypothyroidism
Breast milk

Question 12

What causes physiological jaundice?

Answer 12

High levels of HbF which has a shorter life span
Relative polycythaemia
Relative immaturity of liver function prevents conjugation of bilirubin
Leads to unconjugated jaundice after first day of life

Question 13

What is breast milk jaundice?

Answer 13

Breast fed babies jaundice lasts longer

Question 14

What happens if unconjugated jaundice isn’t prevented?

Answer 14

Kernicterus

Question 15

What is kernicterus?

Answer 15

Unconjugated jaundice can pass the blood brain barrier so toxic deposits in brain

Question 16

How is unconjugated jaundice treated?

Answer 16

Phototherapy - converts photoisomerization of bilirubin to water soluble form

Question 17

What wavelength is used for phototherapy?

Answer 17

450 nanometres

Question 18

What are causes of haeomolysis?

Answer 18

ABO incompatibility
Rhesus disease
Bruising/cephalhaematoma
Red cell membrane defects
Red cell enzyme defects

Question 19

What are causes of abnormal bilirubin conjugation?

Answer 19

Gilbert’s disease

Crigler-Naijar syndrome

Question 20

When is jaundice considered prolonged?

Answer 20

Jaundice beyond 2 weeks of life

Beyond 3 weeks in preterms

Question 21

What are causes of prolonged jaundice?

Answer 21

Biliary obstruction
Neonatal hepatitis
Hypothyroidism
Breast milk jaundice

Question 22

Is there a normal cause of conjugated jaundice in infants?

Answer 22

No - any infant with conjugated jaundice requires investigation

Question 23

What is the most important test in prolonged jaundice?

Answer 23

Split bilirubin

Question 24

In babies with prolonged jaundice what should be examined in stool?

Answer 24

Colour

Question 25

What is biliary atresia?

Answer 25

Congenital fibro-inflammatory disease of bile ducts leading to destruction of extra hepatic bile ducts

Question 26

What are features of biliary atresia?

Answer 26

Prolonged conjugated jaundice Pale stools, dark urine Progression to liver failure if not identified and treated

Question 27

What is the primary treatment for biliary atresia?

Answer 27

Kasai procedure - surgical (Kasai portoenterostomy)

Question 28

What is the main reason to assess children with prolonged jaundice?

Answer 28

Diagnosing patients with biliary atresia early

Question 29

When is Kasai procedure mot likely to succeed?

Answer 29

<60 days | Chance of success rapidly drops after this point

Question 30

What investigations should be done in prolonged conjugated jaundice?

Answer 30

Split bilirubin Stool colour Ultrasound Liver biopsy

Question 31

What are causes of neonatal hepatitis?

Answer 31

``` Alpha-1-antitrypsin deficiency Galactosaemia Tyrosinaemia Urea cycle defects Hypothyroid Viral hepatitis ```