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Paediatrics > Jaundice - yellow baby > Flashcards

Jaundice - yellow baby Flashcards

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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 ?

Study These Flashcards
A

Always pathological

Causes: Haemolysis, Sepsis

23
Q

Intermediate (24hrs – 2 weeks)

jaundice causes?

Study These Flashcards
A

Physiological, Breast milk, Sepsis, Haemolysis, infection

24
Q

Prolonged infant jaundice causes ?

Study These Flashcards
A

(>2 weeks)

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

25
physiological jaudice.. why?
RBC have a shorter life span - have fetal haemoglobin - Relative polycythaemia Relative immaturity of liver function
26
Physiological jaundice is..
pre-hepatic unconjugated - develops after first day of life
27
Great milk jaundice is..
unconjugated
28
unconjugated bilirubin is neurotoxic.. how?
fat-soluble (water insoluble) so can cross blood-brain barrier
29
early features of Kernicterus
encephalopathy – poor feeding, lethargy, seizures
30
late consequences of Kernicterus?
severe choreoathetoid cerebral palsy, learning difficulties, sensorineural deafness
31
Treatment for unconjugated jaundice?
Phototherapy - visible light - not UV!!
32
what does visible light covert in phototherapy?
bilirubin to water soluble isomer (photoisomerisation) | - excreted through the kidneys
33
Other Causes of Early/Intermediate Unconjugated infant jaundice? (Abnormal conjugation)
Sepsis - Gilbert’s disease – common, mild Crigler-Najjar syndrome – v. rare, severe
34
Causes of Haemolysis in jaundice?
``` ABO incompatibility Rhesus disease Bruising/cephalhaematoma Red cell membrane defects (e.g. spherocytosis) Red cell enzyme defects (e.g. G6PD) ```
35
Crigler-Najjar syndrome
complete absence of UDP UDP glucuronyl transferase | - impaired conjugation of bilirubin
36
what tests for severe uncomplicated jaundice?
urine + blood cultures, TORCH screen) Blood group, DCT Examination Blood film G6PD assay more complicated - genotype/phenotype
37
what is prolonged infant jaundice?
2 weeks of life | 3 weeks for preterm infants
38
conjugated causes for prolonged infant jaundice?
``` Anatomical (biliary obstruction) Neonatal hepatitis (liver inflammation) ```
39
unconjugated causes for prolonged infant jaundice?
Hypothyroidism | Breast-milk jaundice
40
most important test in prolonged jaundice??
split bilirubin | - to see if it is conjugated or unconjugated
41
conjugated jaundice in infants is always?
abnormal , require further investigation
42
causes of blurry obstruction? - Biliary atresia
blockage of bile out of liver | - Conjugated jaundice, pale stools
43
Choledochal cyst features
turbulent flow, blockage to bile flow | Conjugated jaundice, pale stools
44
Alagille syndrome features
intra hepatic block - genetic - Intrahepatic cholestasis, dysmorphism, congenital cardiac disease
45
most important cause of prolonged jaundice (conjugated)
Biliary atresia
46
What is Biliary atresia
Congenital fibro-inflammatory disease of bile ducts leading to destruction of extra-hepatic bile ducts - fibrosis and scar tissue
47
how will infants present with Biliary atresia?
Biliary atresia - can lead to liver failure
48
Most common indication for liver transplant
Biliary atresia
49
Treatment for Biliary atresia
Kasai portoenterostomy - best if performed before 60 days (9 weeks)
50
Investigations for bilary atresia?
split bilirubin, stool colour, ultrasound, liver biopsy
51
Investigations for Choledochal cyst?
split bilirubin, stool colour, ultrasound
52
Investigations for Alagille syndrome?
dysmorphism, genotype | cardiac abnormalities?
53
causes of prolonged jaundice – neonatal hepatitis
``` 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
Key tests to differentiate the cause of a neonatal hepatitis picture?
``` 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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