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Paediatrics > Paediatric Jaundice and Metabolism > Flashcards

Paediatric Jaundice and Metabolism Flashcards

1
Q

what are the main functions of the liver?

A
  • toxin excretion
  • nutrient storage
  • production of clotting factors & plasma proteins
  • nutrient metabolism
  • bile production
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2
Q

which liver function tests assess liver damage?

A
  • aminotransferases (AST/ALT)
  • ALP
  • gamma GT
  • bilirubin
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3
Q

what does split bilirubin measure?

A

conjugated (direct) and unconjugated (indirect) bilirubin

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4
Q

why is ALP not often used as a marker for liver damage in children?

A

because ALP is also found in bone, and growing bone in children will produce higher levels of ALP

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5
Q

what are high levels of aminotransferases indicative of?

A

cell damage

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6
Q

what is raised gamma GT an indicator of?

A

biliary tree damage

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7
Q

what is raised ALP an indicator of in liver disease?

A

biliary tree damage

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8
Q

what are raised aminotransferases an indicator of in liver disease?

A

hepatocellular damage

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9
Q

which raised aminotransferase is normally specific to liver cell damage?

A

ALT

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10
Q

which raised aminotransferase can be an indicator of muscle cell damage?

A

AST

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11
Q

which liver function tests indicate damage to the biliary tree?

A

ALP

gamma GT

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12
Q

which lab tests assess liver function?

A
  • coagulation factors (PT/INR/APTT)
  • albumin
  • bilirubin
  • blood glucose and ammonia in severe liver disease
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13
Q

how do children with liver disease present?

A

with jaundice

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14
Q

how can you differentiate between jaundice and beta carotenaemia?

A

jaundice also affects sclera, beta carotenaemia only affects skin

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15
Q

what is the main difference in presentation of chronic liver disease in children vs adults?

A

in children liver disease also presents with growth failure

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16
Q

what is the average level of bilirubin above which infants become jaundiced?

A

> 40-50umol/l

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17
Q

describe bilirubin metabolism

A
  • RBC broken down by reticuloendothelial system (haem + globin)
  • haem becomes biliverdin
  • biliverdin becomes bilirubin (unconjugated - UC)
  • UC bilirubin is transported to liver by albumin
  • glucuronic acid in liver conjugates bilirubin
  • conjugated bilirubin enters small intestine as urobilinogen
  • urobilinogen goes one of there ways : 1. back to liver; 2. excreted by kidneys; 3. excreted in stool as stercobilin
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18
Q

in pre-hepatic jaundice, is bilirubin conjugated or unconjugated?

A

mostly unconjugated

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19
Q

in intrahepatic jaundice, is bilirubin unconjugated or conjugated?

A

mixed unconjugated and conjugated

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20
Q

in post-hepatic jaundice, is bilirubin unconjugated or conjugated?

A

mostly conjugated

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21
Q

why is it important to know the age of a child with jaundice?

A

because their age can indicate the most likely cause for the jaundice

22
Q

what are the three classifications of jaundice by age?

A

early (<24hours old)
intermediate (24 hours - 2 weeks)
prolonged (>weeks)

23
Q

when does physiological jaundice normally present?

A

after first day of life

24
Q

what causes physiological jaundice?

A
  • infants are polycythaemic
  • short RBC half life
  • liver not fully developed, so bilirubin conjugation takes longer
25
Q

which type of jaundice is always abnormal in infants?

A

early jaundice - if less than 24 hours old

26
Q

name a few causes of early neonatal jaundice

A

sepsis

haemolysis

27
Q

what type of jaundice can be seen as a result of breast milk?

A

prolonged jaundice - unconjugated

28
Q

name a few causes of prolonged neonatal jaundice

A
  • extrahepatic obstruction
  • neonatal hepatitis
  • hypothyroidism
  • breast milk
29
Q

what type of jaundice is physiological jaundice in infants?

A

intermediate jaundice, unconjugated

30
Q

what are the two conjugation disorders which can cause early/intermediate jaundice in infants?

A

Gilbert’s syndrome (mild)

Crigler-Najjar syndrome (severe)

31
Q

despite physiological jaundice not being dangerous, what important complication needs to be avoided?

A

kernicterus

32
Q

what is kernicterus?

A

build up of unconjugated bilirubin in basal ganglia, resulting in encephatlopathy, learning difficulties, sensorineural deafness, choreoathetoid cerebral palsy

33
Q

why can physiological jaundice cause kernicterus in infants?

A

because in physiological jaundice bilirubin is unconjugated therefore fat soluble = it can cross the BBB

34
Q

how is unconjugated infant jaundice treated?

A

phototherapy - converts unconjugated bilirubin into a water soluble isomer

35
Q

why can infants present with haemolysis resulting in early jaundice?

A
  • hereditary red cell abnormalities (eg spherocytosis, G6PD)

- blood type incompatibility (ABO, rhesus)

36
Q

what is the most important cause for prolonged conjugated infant jaundice?

A

biliary atresia

37
Q

what is biliary atresia?

A

congenital auto-immune condition leading to fibrosis/destruction of biliary tree

38
Q

what is prolonged infant jaundice?

A

jaundice lasting beyond 2 weeks of life

39
Q

if an infant presents with unconjugated prolonged jaundice, what are the likely causes?

A

breast milk

hypothyroidism

40
Q

what is the most crucial test to do in infants presenting with prolonged jaundice?

A

split bilirubin

41
Q

what clinical sign should never be missed in infants presenting with prolonged jaundice?

A

pale stools - steatorrhea

42
Q

what is the appearance of stool and urine in biliary atresia?

A

dark urine

pale stool

43
Q

what is the surgical treatment of biliary atresia?

A

Kasai portoenterostomy

44
Q

when is a Kasai portoenterostomy beneficial in infants with biliary atresia?

A

if they are under 2 months old

45
Q

if an infant with biliary atresia is older than 2 months, what is the best management option?

A

waiting list for liver transplant

46
Q

what is the main reason for assessing infants with prolonged jaundice?

A

for early detection and treatment of biliary atresia

47
Q

what investigations are done to diagnose biliary atresia?

A
  • split bilirubin
  • stool sample
  • ultrasound
  • liver biopsy
48
Q

which types of infant jaundice are always pathological?

A
  • early infant jaundice

- prolonged conjugated jaundice

49
Q

name a few causes of neonatal hepatitis

A 
anything that causes liver inflammation:
alpha 1 anti trypsin deficiency
galactosaemia
hypothyroidism
haemochromatosis
viral hepatitis
urea cycle defects
glycogen storage disorders
50
Q

how does alpha 1 anti trypsin deficiency affect children vs adults?

A

children: liver damage (hepatitis)
adults: lung damage (emphysema)

Paediatrics (10 decks)

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