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Paediatrics > Jaundice - yellow baby > Flashcards

Jaundice - yellow baby Flashcards

1
Q

What makes up LFT’S

A

Bilirubin
Total bilirubin
“Split” bilirubin – Direct (conjugated) + Indirect (unconjugated)

ALT/AST (alanine aminotransferase/aspartate aminotransferase)

Alkaline phosphatase

Gamma glutamyl transferase (GGT)

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2
Q

The liver functions

A

clotting factors

  • absorption, digestion and metabolism of food types
  • bile absorbs fat
  • cabrohydrates and proteins
  • storage organ - glycogen = glucose
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3
Q

what is split bilirubin - direct and indirect

A

(conjugated) + Indirect (unconjugated)

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4
Q

ALT/AST is elevated in?

A

hepatocellular damage (“hepatitis”)

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5
Q

what is elevated in biliary ideas?

A

Alkaline phosphatase

Gamma glutamyl transferase (GGT)

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6
Q

How to assess the function of the liver? (5)

A 
Coagulation - clotting function
Prothrombin time (PT)/INR
APTT

Albumin - how is the liver producing protein

Bilirubin

(Blood glucose)

(Ammonia)

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7
Q

how does liver disease present in children?

A

JAUNDICE

Incidental finding of abnormal blood test

Symptoms/signs of chronic liver disease

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8
Q

signs of chronic liver disease?

A 
growth failure
jaundice 
epistaxis 
encephalopathy 
spider naevi
portal hypertension - varices 
bruising with petechiae 
splenomegaly
ascites
clubbing 
ricks - vit D
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9
Q

Signs of cholestasis?

A 
fat malabsorption 
deficiency of fat soluble vitamins
pruritus
pale stools
dark urine
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10
Q

when is Jaundice visible

A

total bilirubin >40-50 umol/l

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11
Q

where to pick up signs of jaundice

A

sternum - yellow tinge

sclera

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12
Q

beta carotenemia only discolours the?

A

skin

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13
Q

biliverdin is converted to?

A

Unconjugated bilirubin = bound to albumin

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14
Q

what acid is used in Conjugation ?

enzyme?

A

glucuronic acid

UDP glucuronyl transferase

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15
Q

Conjugated bilirubin goes on to become

A

bile -

small intestine = Urobilinogen

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16
Q

Urobilinogen is excreted by?

A

kidneys as urine

reabsorbed in the hepatic circulation
or as

Stercobilin (faecal pigment)
Excreted in stool

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17
Q

unconjucated bilirubin is?

A

water insoluble - needs transported bound to albumin

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18
Q

conjugated bilrubin is?

A

water soluble

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19
Q

what is pre- hepatic jaundice

A

cause of jaundice where propel is before the liver

  • too much bilirubin
  • increased break down of RBC
  • UNCONJUCATED
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20
Q

What is Intrahepatic jaundice

A

mixed unconjugated/conjugated

  • liver not functioning well
  • oedema and hold up
  • not excreting into the bile
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21
Q

what is Post-hepatic jaundice (cholestasis)

A

mostly conjugated elevation

  • bile doesn’t get out of the liver
  • obstructive process
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22
Q

causes for jaundice in the first 24 hours ?

A

Always pathological

Causes: Haemolysis, Sepsis

23
Q

Intermediate (24hrs – 2 weeks)

jaundice causes?

A

Physiological, Breast milk, Sepsis, Haemolysis, infection

24
Q

Prolonged infant jaundice causes ?

A

(>2 weeks)

Causes: Extrahepatic obstruction, Neonatal hepatitis, Hypothyroidism, Breast milk

25
Q

physiological jaudice.. why?

A

RBC have a shorter life span
- have fetal haemoglobin
- Relative polycythaemia
Relative immaturity of liver function

26
Q

Physiological jaundice is..

A

pre-hepatic
unconjugated
- develops after first day of life

27
Q

Great milk jaundice is..

A

unconjugated

28
Q

unconjugated bilirubin is neurotoxic.. how?

A

fat-soluble (water insoluble) so can cross blood-brain barrier

29
Q

early features of Kernicterus

A

encephalopathy – poor feeding, lethargy, seizures

30
Q

late consequences of Kernicterus?

A

severe choreoathetoid cerebral palsy, learning difficulties, sensorineural deafness

31
Q

Treatment for unconjugated jaundice?

A

Phototherapy - visible light - not UV!!

32
Q

what does visible light covert in phototherapy?

A

bilirubin to water soluble isomer (photoisomerisation)

- excreted through the kidneys

33
Q

Other Causes of Early/Intermediate Unconjugated infant jaundice?
(Abnormal conjugation)

A

Sepsis
- Gilbert’s disease – common, mild
Crigler-Najjar syndrome – v. rare, severe

34
Q

Causes of Haemolysis in jaundice?

A 
ABO incompatibility
Rhesus disease
Bruising/cephalhaematoma
Red cell membrane defects (e.g. spherocytosis)
Red cell enzyme defects (e.g. G6PD)
35
Q

Crigler-Najjar syndrome

A

complete absence of UDP UDP glucuronyl transferase

- impaired conjugation of bilirubin

36
Q

what tests for severe uncomplicated jaundice?

A

urine + blood cultures, TORCH screen)

Blood group, DCT
Examination
Blood film
G6PD assay

more complicated - genotype/phenotype

37
Q

what is prolonged infant jaundice?

A

2 weeks of life

3 weeks for preterm infants

38
Q

conjugated causes for prolonged infant jaundice?

A 
Anatomical (biliary obstruction)
Neonatal hepatitis (liver inflammation)
39
Q

unconjugated causes for prolonged infant jaundice?

A

Hypothyroidism

Breast-milk jaundice

40
Q

most important test in prolonged jaundice??

A

split bilirubin

- to see if it is conjugated or unconjugated

41
Q

conjugated jaundice in infants is always?

A

abnormal , require further investigation

42
Q

causes of blurry obstruction? - Biliary atresia

A

blockage of bile out of liver

- Conjugated jaundice, pale stools

43
Q

Choledochal cyst features

A

turbulent flow, blockage to bile flow

Conjugated jaundice, pale stools

44
Q

Alagille syndrome features

A

intra hepatic block

  • genetic
  • Intrahepatic cholestasis, dysmorphism, congenital cardiac disease
45
Q

most important cause of prolonged jaundice (conjugated)

A

Biliary atresia

46
Q

What is Biliary atresia

A

Congenital fibro-inflammatory disease of bile ducts leading to destruction of extra-hepatic bile ducts
- fibrosis and scar tissue

47
Q

how will infants present with Biliary atresia?

A

Biliary atresia - can lead to liver failure

48
Q

Most common indication for liver transplant

A

Biliary atresia

49
Q

Treatment for Biliary atresia

A

Kasai portoenterostomy - best if performed before 60 days (9 weeks)

50
Q

Investigations for bilary atresia?

A

split bilirubin, stool colour, ultrasound, liver biopsy

51
Q

Investigations for Choledochal cyst?

A

split bilirubin, stool colour, ultrasound

52
Q

Investigations for Alagille syndrome?

A

dysmorphism, genotype

cardiac abnormalities?

53
Q

causes of prolonged jaundice – neonatal hepatitis

A 
CONJUGATED 
Alpha-1-antitrypsin deficiency
Galactosaemia
Tyrosinaemia
Urea cycle defects
Haemochromatosis
Glycogen storage disorders
Hypothyroidism
Viral hepatitis - HEP B, C, uti, septicemia, EBV, meningitis 
Parenteral nutrition
54
Q

Key tests to differentiate the cause of a neonatal hepatitis picture?

A 
phenotype/level
GAL-1-PUT
amino acid profile
ammonia
iron studies, liver biopsy
biopsy
TFTs
serology, PCR
history

Paediatrics (49 decks)

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