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Paediatrics > Paediatric Liver and Infant Nutrition > Flashcards

Paediatric Liver and Infant Nutrition Flashcards

1
Q

How do signs of chronic liver disease alter in paediatrics compared to adult?

A

Same signs but additionally there is growth failure

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2
Q

Where is it best to check for jaundice and why?

A

The sclera
Yellowing of the skin such as beta carotenaemia can occur but will not affect the sclera so sclera good for differentiating

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3
Q

Briefly describe the metabolism of bilirubin

A

Post-mature erythrocytes are broken down to unconjugated bilirubin, which is transported to the liver where it is conjugated. It is then excreted through the bile into the small intestine and passed through the stool

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4
Q

How does a split bilirubin help narrow down the location of the cause?

A

Split bilirubin gives levels of unconjugated and conjugated bilirubin separately.
•Mainly unconjugated bilirubin- pre-hepatic
•Mixed unconjugated and conjugated- hepatic
•Mainly conjugated- post-hepatic

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5
Q

What are the causes of early jaundice in the first 24hrs of life?

A

Always pathological, usually caused by haemolysis or sepsis

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6
Q

What is the definition of intermediate jaundice?

A

Intermediate jaundice is jaundice between 24hrs and 2 weeks of life

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7
Q

What are the causes of intermediate jaundice?

A

Physiological
Breast milk
Sepsis
Haemolysis

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8
Q

What is the definition of prolonged jaundice?

A

Jaundice occurring from two weeks of life onwards or three weeks in preterm infants

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9
Q

What are the causes of prolonged jaundice?

A

Extrahepatic obstruction
Neonatal hepatitis
Hypothyroidism
Breast milk jaundice

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10
Q

What is physiological jaundice?

A

Unconjugated jaundice that onsets after the first day of life

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11
Q

What are the causes of physiological jaundice?

A

Shorter red blood cell lifespan in infants
Relative polycythaemia
Relative immaturity of liver function

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12
Q

What is breast-milk jaundice?

A

Unconjugated jaundice that persists in breast-fed infants for up to 12 weeks for unknown reasons

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13
Q

What are the other causes of early/intermediate unconjugated infant jaundice other than physiological or breast fed?

A

Sepsis
Haemolysis- ABO incompatibility, rhesus disease, red cell defects
Abnormal conjugation- Gilbert’s disease (common, mild), Crigler-Najjar syndrome (very rare but serious)

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14
Q

What tests can be useful in investigating causes of early/intermediate jaundice?

A 
Urine + blood cultures
TORCH screen
Blood group
Direct Coombs test
Blood film
G6PD assay
Genotype/phenotype testing
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15
Q

What is kernicterus?

A

An important complication of early infant jaundice

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16
Q

Why does kernicterus develop?

A

As unconjugated bilirubin is fat soluble it can cross the blood-brain barrier and leave neurotoxic deposits in the brain. It can be a complication of any kind of jaundice, even physiological

17
Q

What are the early signs of kernicterus?

A

Encephalopathy related- poor feeding, lethargy and seizures

18
Q

What are the late signs of kernicterus?

A

Severe choreoathetoid cerebral palsy
Learning difficulties
Sensorineural deafness

19
Q

How is unconjugated jaundice treated?

A

Phototherapy using visible light of a 450nm wavelength that converts bilirubin to a water soluble isomer

20
Q

What are the causes of prolonged jaundice?

A 
Conjugated:
•Anatomical- biliary obstruction
•Neonatal hepatitis
Unconjugated:
•Hypothyroidism
•Breast-milk jaundice
21
Q

What biliary obstructions can cause prolonged jaundice?

A

Biliary atresia- conjugated jaundice + pale stools
Choledochal cyst- conjugated jaundice + pale stools
Alagille syndrome- intrahepatic cholestasis, dysmorphism, congenital cardiac disease

22
Q

What additional step should be taking when assessing infant jaundice?

A

Always assess stool colour

23
Q

What is biliary atresia?

A

A congenital fibro-inflammatory disease of the bile ducts that leads to destruction of the extra-hepatic bile ducts

24
Q

What are the symptoms of biliary atresia?

A

Patients present with prolonged, conjugated jaundice and will have pale stools and dark urine. It will progress to liver failure if it is not identified and treated

25
Q

How is biliary atresia treated?

A

First line treatment is a Kasai portoenterostomy but success rate diminishes rapidly with age and if >9 weeks old then a liver transplant should be done instead

26
Q

How is biliary atresia investigated?

A

Split bilirubin
Stool colour
Ultrasound
Liver biopsy

27
Q

How is a choledochal cyst investigated?

A

Split bilirubin
Stool colour
Ultrasound

28
Q

How is Alagille syndrome investigated?

A

Dysmorphism

Genotype

29
Q

What are the causes of neonatal hepatitis?

A 
Alpha-1-antitrypsin deficiency
Galactosaemia
Tyrosinaemia
Urea cycle defects
Haemochromatosis
Glycogen storage disorders
Hypothyroidism
Viral hepatitis
Parenteral nutrition
30
Q

What are the phases of growth?

A

Infant- nutrition led
Child- growth hormone led
Pubertal- sex steroid led

31
Q

Why do babies have a high energy requirement?

A

High energy demand for thermogenesis, physical activity, tissue maintenance and growth
Growth demands- 35% of energy intake in infants

32
Q

How does average weight gain occur in infants?

A

0-3 months 200g/week
3-6 months 150g/week
6-9 months 100g/week
9-12 months 50-75g/week

33
Q

How does children’s growth occur from one year onwards?

A

After 1 year there is approximately 2kg and 5cm growth per year until puberty

34
Q

What are the benefits of breast feeding?

A

Suckling/bonding
Good nutrition for 6 months for most infants
Passive immunity
Encourages development of infant’s active immunity
Encourages development of infant’s gut mucosa
Reduced infection
Antigen load minimal

35
Q

What are the pros and cons of formula feed?

A 
No anti-infection properties
Risk of contamination
No transition of BBVs/drugs
High antigen load
Expensive
Doesn’t need mum
Accurate feed volumes
Provides vit K
Less jaundice
36
Q

What replacement feeds can be given to infants with a cows milk allergy?

A

Extensively hydrolysed protein feeds are the first line choice
Amino acid based feeds are the second line choice

37
Q

What is weaning and when does it occur?

A

Weaning is the transition from a milk based diet to a mixed diet. It starts at 5-6 months with smoot purees, introducing lumps/finger food from 6-7 months

Paediatrics (22 decks)

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