Gastroenterology 4

Question 1

What causes jaundice?

Answer 1

over 50% of all newborn infants become visibly jaundiced because of:
• marked physiological release of Hb from the breakdown of RBCs, because of high Hb
concentration at birth
• markedly shorter red cell lifespan of newborn infants (~ 70 days) compared to that
of adults (~ 120 days)
• hepatic bilirubin metabolism being less efficient in the first few days of life
Neonatal jaundice is important as it may be sign of another disorder and unconjugated bilirubin can be deposited in the brain, particularly in the basal ganglia- kernicterus

Question 2

What are the risk factors for significant hyperbillirubinaemia?

Answer 2

gestational age under 38 weeks
• older sibling with neonatal jaundice requiring phototherapy
• mother’s intention to breastfeed exclusively
• visible jaundice in the first 24h of life
Babies become clinically jaundiced when bilirubin =< 80 umol/l

Question 3

What causes jaundice <24hrs of age (haemolytic disorders?

Answer 3

Rhesus haemolytic disease
ABO incompatibility
G6PD deficiency (precipitated by drugs in later life)
Spherocytosis

Question 4

What causes jaundice at 24h-2 weeks of age?

Answer 4

Physiological jaundice 
Breast milk jaundice
Infection (UTI)
Haemolysis 
Polycythaemia 
Crigler-Najjar syndrome

Question 5

What causes unconjugated jaundice at 2 weeks?

Answer 5

Breast milk jaundice
Infection 
Hypothyroidism 
Haemolytic anaemia 
High GI obstruction (pyloric stenosis)

Question 6

What causes conjugates (>25umol/l) jaundice at 2 weeks of life?

Answer 6

Neonatal hepatitis

Bile duct obstruction

Question 7

What are the investigations for jaundice?

Answer 7

do not measure bilirubin levels routinely in babies who aren’t visibly jaundiced If baby is red, you can blanch the skin to see if it’s yellow
Jaundice <24h: CRP, FBC, SBR, blood group, Coombs +/- septic screen
Jaundice >24h: SBR, other’s if clinically indicated
Jaundice >14 days: SBR, urine testing, TFTs. If total/conjugated bilirubin >25% (which is significant): further Ix

Question 8

What should be done in babies with suspected or obvious jaundice?

Answer 8

Examine the baby carefully under bright light.
Make sure to inspect sclera and gums
Use SBR in <24hr or GA < 35/40. In all other babies use transcutaneous bilirubinometer (if it shows levels greater than 250 umol/l, measure serum bilirubin)

Question 9

How do you assess for underlying disease in jaundice?

Answer 9

Serum bilirubin (for baseline level to assess
response to treatment)
o blood packed cell volume
o blood group (maternal & the baby)
o Coomb’s test to check for ABO incompatibility
o FBC + blood film examination
o Blood G6PD levels, if indicated by ethnic origin (more common in
Mediterranean, Middle-East or African people) o Blood/urine/CSF culture, if infection suspected

Question 10

What is the management for jaundice?

Answer 10

Phototherapy- 450nm waves from the blue-green spectrum convert unconjugated bilirubin into a harmless water-soluble pigment excreted predominantly in urine
Continuous intensive therapy is given if bilirubin is rising rapidly or has reached high levels

Question 11

What are the side effects of phototherapy?

Answer 11

Temperature instability (as the infant is undressed), macular rash and bronze discoloration of the skin if the jaundice is conjugated

Question 12

What is exchange transfusion?

Answer 12

Used for severe cases
Blood is removed from the baby in small portion from an arterial line or umbilical vein and replaced with donor blood via peripheral or umbilical vein. Usually twice the infant’s blood volume (2 x 90 ml/kg) is exchanged

Question 13

What is biliary atresia?

Answer 13

Occurs in 1 in 15 000 live births. There is progressive fibrosis and obliteration of the extrahepatic and intrahepatic biliary tree. Without intervention causes chronic liver failure and death within 2 years

Question 14

How does biliary atresia present?

Answer 14

Mild jaundice and (progressively) pale stools. Faltering growth usually occurs and hepatosplenomegaly may develop secondary to portal hypertension

Question 15

What are the investigations for biliary atresia?

Answer 15

there is raised conjugated bilirubin and abnormal LFTs. Liver biopsy initially
demonstrates neonatal hepatitis with extrahepatic obstruction features developing
later
• a fasting abdominal USS may demonstrate a contracted or absent gallbladder, but it
may also be normal
Radioisotope scan with TIBIDA shows good uptake in the liver, but no excretion into the bowel
• diagnosis is confirmed by an CERCP, which fails to outline a normal biliary tree

Question 16

What is the management for biliary atresia?

Answer 16

Palliative Kasai hepatoportoenterostomy
(attaching loop of jejunum to porta hepatis, bypassing the malfunctioning duct). Early surgery increases jaundice clearing rate
If Kasai surgery fails, liver transplantation is considered.
In either case, nutrition and fat-soluble vitamin supplementation is essential.

Question 17

What is the prognosis of biliary atresia?

Answer 17

Even with successful clearance of jaundice, the disease progresses in most children who may develop cholangitis and cirrhosis with portal hypertension

Question 18

What are the NICE recommendations for breast feeding?

Answer 18

should be done exclusively for the first 6 months of life

Feed should be given within the first hour of life along with baby to mother skin to skin contact

Question 19

What are the advantages of breast feeding?

Answer 19

o Ideal nutrition- life-saving in developing countries
o Reduces GI infection and necrotising enterocolitis (preterm)
o Enhances relationship
o Reduces the risk of insulin dependent diabetes, hypertension and obesity later in life
o Reduction in breast cancer risk in the mother

Question 20

What are the potential complications of breast feeding?

Answer 20

o	An unknown quantity is taken each time
o	Transmission of some diseases
o	Transmission of drugs and environmental contaminants
o	Less flexible than formula feeding
o	Nutrient inadequacies
o	Risk of breast-milk jaundice

Question 21

What is kernicterus?

Answer 21

Encephalopathy resulting from deposition of unconjugated bilirubin in the basal ganglia and brainstem nuclei
Occurs when unconjugated bilirubin levels exceed albumin-binding capacity
Lethargy and poor feeding
Severe- irritability, increased muscle tone, seizures and coma
Infants who survive may develop choreoathetoid cerebral palsy , learning difficulties and sensorineural deafness