paediatric liver Flashcards

1
Q

1.

Which LFT is raised in hepatocellular dameage

A

ALT

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2
Q

Which LFTs are raised in biliary disease

A

ALP and GGT

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3
Q

What is the best way to assess liver function

A

Check the prothrombin time or the INR

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4
Q

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

A

Jaundice

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5
Q

What is the bilirubin level in jaundice

A

Usually greater than 40-50 micromol/l

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6
Q

What is conjugated bilirubin

A

Glucoronic acid gets added to the bilirubin in the liver

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7
Q

Which bilirubin is water soluble

A

Conjugated is soluble in water - unconjugated is insoluble in water

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8
Q

What kind of bilirubin is associated with prehepatic jaundice

A

Mostly unconjugated

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9
Q

What kind of bilirubin is associated with intra-hepatic jaundice

A

mixed - conjugated and unconjugated

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10
Q

What kind of bilirubin is associated with post-hepatic jaundice

A

Mostly conjugated bilirubin

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11
Q

What causes of jaundice are suspected when the baby is less than 24 hours old

A

Haemolysis or sepsis

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12
Q

What causes of jaundice are suspected in a child who is between 24 hours and 2 weeks old

A
  • Breast milk
  • sepsis
  • haemolysis
  • physiological
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13
Q

What causes of jaundice are suspected in children over 2 weeks old

A
  • Extra-hepatic obstruction
  • Neonatal hepatitis
  • Hypothyroidism
  • Breast milk
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14
Q

Describe physiological jaundice

A

Unconjugated jaundice which develops after the first day of life

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15
Q

What is the cause of physiological jaundice

A

Infants have a shorter red blood cell life span and are born with high blood cell count with immature liver function which can lead to jaundice due to excess red blood cell break down

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16
Q

Describe breast milk jaundice

A

Unconjugated jaundice that can persist up to 12 weeks

17
Q

What is kernicterus

A

Unconjugated bilirubin crossing the blood brain barrier due to it being fat solube

18
Q

What is the presentation of kernicterus

A
  • Encephalopathy (poor feeding, lethargy and seizures)
  • Severe choreoathetoid cerebral palsy, learning difficulties and sensorineural deafness
19
Q

What is the treatment for unconjugated jaundice

A

Phototherapy

20
Q

Describe phototherapy

A

Visible light which converts bilirubin to water soluble isomer which makes it unable to cross the blood brain barrier

21
Q

Describe gilberts disease

A

Slight reduction of the UDP glucuronyl transferase and therefore glucuronic acid cannot get added to bilirubin and therefore the bilirubin stays unconjugated

22
Q

Describe Crigler-Najjar syndrome

A

almost no UDP glucuronyl transferase and therefore glucuronic acid cannot be added to bilirubin which therefore leads to more unconjugated bilirubin - more severe version of gilberts disease

23
Q

Define prolonged infant jaundice

A

Jaundice that persists beyond 2 weeks of life

24
Q

What are the causes of prolonged infant jaundice

A

Conjugated:
- Anatomical biliary obstruction
- Neonatal hepatitis
Unconjugated:
- hypothyroidism
- Breast milk jaundice

25
What is the most important test in prolonged jaundice in an infant and why
Split bilirubin test to find out whether the jaundice is conjugated or unconjugated
26
What does biliary atresia present with
Prolonged obstructive jaundice - conjugated jaundice - pale stools - dark urine
27
What does a choledochal cyst present with
Conjugated jaundice and pale stools + dark urine
28
What is the presentation of alagille syndrome
- Intrahepatic cholestasis - Dysmorphism - congenital cardiac disease
29
What does a pale stool in an infant indicate
Obstructive jaundice
30
What is biliary atresia
Congenital fibro-inflammatory disease of bile ducts which leads to the destruction of the extra-hepatic bile ducts - cuases prolonged jaundice (obstructive)
31
What is the treatment for biliary atresia
Kasai portoenterostomy - the fibrosed bile ducts are removed and then the small intestine is anastamosed onto the liver surface so the bile directly goes to the small intestine
32
What is conjugated jaundice diagnosis until proven otherwise in infants
Biliary atresia