paediatric infant and liver nutrition

Question 1

Liver function tests

Answer 1

Bilirubin

• Total
• split conjugated and unconjugated

AST/ALT

Alkaline phosphatase

Gamma glutamyl transferase

Question 2

test to assess liver function

Answer 2

coagulation
Albumin
Bilirubin
Blood glucose-
Ammonia- Things go wrong in liver dysfunction

Question 3

Signs of chronic liver disease in children

Answer 3

+growth failure

Question 4

infant jaundice

Answer 4

usually visible when bilirubin >40-50umol/l

Question 5

understanding bilirubin metabolism

age of infant

Answer 5

diagnosis of infant jaundice

Question 6

metabolism

Answer 6

post mature erythrocytes-> Haem-> bilverdin-> unconjugated bilirubin-bound to albumin-> transported to liver->conjugation-> conjugated bilirubin->bile->small intestine->urobilinogen->stercobilin

conjugated-water soluble

Question 7

pre hepatic

Answer 7

mostly unconjugated

Question 8

age of infant

Answer 8

Early (<24hrs old)
always pathological
causes: haemolysis, sepsis

Intermediate (24hrs-2 weeks)
causes: physiological, breast milk, sepsis

prolonged (>2 weeks)
causes: extrahepatic obstruction

Question 9

physiological jaundice

Answer 9

shorter RBC life span in infants
Relative polycythaemia
relative immaturity of liver function

unconjugated jaundice
develops after first day of life
prolongation of jaundice in breastfed infants

Question 10

kernitcterus

Answer 10

unconjugated bilirubin is fat soluble to can cross BBB
neurotoxic and deposits in brain
early signs-encephalopathy
late consequence, severe choreoathetoid cerebral palsy

Question 11

phototherapy

Answer 11

treatment for unconjugated jaundice

visible light converts bilirubin to water soluble isomer (photosiomerisation

threshold for ohototherapy in infants guided by charts

Question 12

prolonged infant jaundice

Answer 12

persisting beyond 2 weeks
-3 weeks for preterm infants

causes:
anatomical(biliary obstruction)
neonatal hepatitis
hypothyroidism
breast milk jaundice

Question 13

causes of prolonged jaundice

Answer 13

biliary atresia-conjugated jaundice , pale stools
choledosyal cyst

always assess stool colour in infants with prolonged jaundice

Question 14

Biliary atresia

Answer 14

congenital fibro inflammatory destruction of bile ducts leading to destruction of extra hepatic bile ducts

present prolonged, conjugated jaundice
pale stools, dark urine
progression to liver failure if not indetified and treated
timly diagnosis critical as time determines prognosis
most common indication for liver transplant

treatment:
Kasai portoenterostomy
-small intestine connected to liver
best results if performed within 60 days

Question 15

Choledochal cyst

Answer 15

conjugated, pale stools

Question 16

alagille syndrome

Answer 16

intrahepatic blockage, dysmorphism, genotype

Question 17

summary

Answer 17

always ask about stool colour
most important test is a split bilirubin
conjugated jaundice always requires further investigation and should always consider biliary atresia