Paediatric Liver Disease

Question 1

what LFTs will be elevated in cases of hepatitis?

Answer 1

ALT/AST

Question 2

What will be elevated in billiary disease

Answer 2

Alkaline Phosphatase and GGT

Question 3

Name other Tests to Assess Function of the liver

Answer 3

Coagulation - PT/INR/APTT
Albumin
Bilirubin
(blood Glucose)
(Ammonia)

Question 4

Dx of infant jaundice is dependant on…

Answer 4

Understanding bilirubin metabolism

Age of the infant

Question 5

Classification of neonatal Jaundice by infant age

Answer 5

Early (<24 hours old)
ALWAYS PATHOLOGICAL
Haemolysis/Sepsis

Intermediate (24hrs to 2 weeks)
Physiological, Breat mik, aemolysis, Sepsis

Prolonged (>2 weeks)
Extrahepatic obstruction, neonatal hepatitis, hypothyroidism, Breast milk

Question 6

Features for physiological Jaundice

Answer 6

RBC has shorter life span in infants (80-90 days)
Relative Polycythaemia
Relative immaturity of liver functions

UNCONJUGATED jaundice
Develops >24hr of life

Question 7

Breast milk Jaundice

Answer 7

Also unconjugated
Can Last up to 12 weeks

exact cause unknown

Question 8

Other causes of Early/intermediate unconjugated infant jaundice

Answer 8

Sepsis (urine + bloods and TORCHES screen)
haemolysis
ABO incompatibility (Blood grouping and DCT)
Rhesus disease (blood group and DCT)
Bruising/Cephalhaematoma (examination)
Red cell membrane defect (spherocytosis) (Blood film)
Red cell enzyme defect (G6PD) (G6DP Assay)
Abnormal conjugation (geno/phenotyping)

Question 9

Complications of earu/intermediate unconjugated infant jaundice

Answer 9

Kernicterus -
Unconjugated biliruben is fat soluble so CAN cross the BBB

neurotoxins deposit i the brain
EARLY SIGNS - encephalopathy - Poor feeding, Lethargy, Seizures
LATE CONSEQUENCES - Severe Choreoathetid cerebral Palsy, LD, sesorineural Deafness

Question 10

Tx for unconjugated Biliruben

Answer 10

Light Therapy - Visible light 450nm

light converts biliruben to water soluble photoisomer
be aware of the guidance for thresholds

Question 11

Prolonged infant jaundice

Answer 11

Jaundice that persists for > 3 wees of life (3 in preterm babies)

Question 12

Causes of Prolonged Jaundice

Answer 12

Conjugated Unconjugated
Anatomical (biliary obstruction) Hypohyroidism
Neonatal Hepatits Breast Milk

Question 13

Key messages of Prolonged Jaundice

Answer 13

Conjugated jaundice in infants is ALWAYS abnormal and requires further testing - The most important test in prolonged jaundice is split biliruben

ALWAYS assess stool colour in infants with prolonged biliruben

Assessment of prolonged biliruben is primarily targeted at Dx of biliary atresia as early as possible

Question 14

What is Biliary atresia

Answer 14

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

presents with Prolonged conjugated biliruben

Pale stools and Dark Urine

Progression to liver disease if not treated (most common indication for liver transplant in children)

(Dx with split biliruben Stool colour, USS and Liver Bx)

Question 15

Rx of Biliary Atresia

Answer 15

Kasai portoenterostomy

Success rapidly diminishes with age
best results if performed before 60 days (<9weeks)

Question 16

Other Causes of Prolonged Jaundice

Answer 16

Chledochal cyst - Pale stools, conjugated biliruben (USS)

Alagille syndrome - intraheaptic cholestasis, dysmorphism, congenital cardiac disease

Neonatal Hepatitis