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Paeds > The Yellow Baby > Flashcards

The Yellow Baby Flashcards

1
Q

Function of the liver

A 
Produces Essential proteins
- albumin 
- clotting factors
Absorption and metabolism of food 
- fat
- carbohydrate 
Storage 
- glycogen 
- other substrates and minerals 
Metabolism and excretion of toxins
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2
Q

LFTs

A 
Bilirubin 
- Total 
- Split 
ALT / AST
Alkaline phosphatase
GGT
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3
Q

What is “split bilirubin”?

A

Direct (conjugated) and indirect (unconjugated)

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4
Q

What does bilirubin indicate?

A

How well you liver is clearing bile

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5
Q

What are ALT / AST elevated in?

A 
Hepatocellular damage (hepatitis) 
Other processes e.g. muscle diseases
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6
Q

What are ALT / AST?

A

Transaminases

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7
Q

When is alkaline phosphatase elevated?

A

Biliary disease

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8
Q

Which LFT is usually ignored in children and why?

A

Alkaline phosphate

Because it is affected by bone growth and so can fluctuate and be high but nothing to do with their liver at al

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9
Q

When is GGT elevated?

A

Biliary disease

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10
Q

What does GGT stand for?

A

Gamma glutamyl transferase

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11
Q

Tests to assess liver function

A 
Coagulation 
- prothrombin time PT/INR
- APTT
Albumin 
Bilirubin 
Blood glucose
Ammonia
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12
Q

Presentation of paediatric liver disease

A 
JAUNDICE 
abnormal incidental finding on blood test 
Chronic ; 
Encephalopathy 
Epistaxis 
Spider naevi 
Varices with portal HTN
muscle wasting from malnutrition 
bruising and petechiae 
splenomegaly with portal HTN
Hypersplenism
Hepatorenal failure 
Liver palms 
Clubbing
Loss of fat stores secondary to malnutrition 
Hypotonia 
Ascites
Peripheral neuropathy
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13
Q

What is jaundice?

A

A yellow discolouration of skin and tissues due to accumulation of bilirubin

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14
Q

Where Is jaundice usually most obvious?

A

Sclera

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15
Q

At what level is bilirubin usually visible?

A

> 40 - 50 umol/l

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16
Q

Bilirubin metabolism

A
  1. Break down of RBCs into biliverdin
  2. Converted into unconjugated bilirubin by biliverdin reductase of tissues which happens all over the body
  3. Bilirubin goes to the liver and is converted to conjugated bilirubin - which goes to bile and gallbladder and is converted in small intestine into urobillogen
  4. either excreted by kidneys or going in stercobilin and is excreted into stool
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17
Q

What does stercobilin do?

A

Makes faeces the colour they are

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18
Q

Solubility of unconjugated bilirubin

A

Water insoluble

Fat soluble

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19
Q

Features of unconjugated bilirubin

A

Cant be directly excreted into kidneys or stool

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20
Q

What is neonatal jaundice classified by?

A

Age

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21
Q

What class of neonatal jaundice is ALWAYS pathological?

A

Early ( < 24 hrs old)

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22
Q

Causes of early ( <24 hours old) jaundice

A

Haemolysis

Sepsis

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23
Q

How old is intermediate neonatal jaundice?

A

24 hours - 2 weeks

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24
Q

Causes of intermediate neonatal jaundice

A 
Physiological 
Breast milk 
Sepsis
Haemolysis
Abnormal conjugation of bilirubin
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25
Q

How long is prolonged neonatal jaundice?

A

> 2 weeks old

3 weeks for pre term infants

26
Q

Causes of prolonged neonatal jaundice

A

Extrahepatic obstruction
Neonatal hepatitis
Hypothyroidism
Breast milk

27
Q

Why do infants get neonatal jaundice?

A

Shorter RBC life span in infants (80-90 days)
Relative polycythaemia (180-200)
So loads of unconjugated bilirubin is produced
Relative immaturity of liver function

28
Q

When does physiological jaundice occur?

A

AFTER the first day of life

29
Q

What type of jaundice is physiological jaundice?

A

Unconjugated jaundice

30
Q

What type of jaundice Is breast milk jaundice?

A

Unconjugated jaundice

31
Q

How long can breast milk jaundice persist for?

A

up to 12 weeks

32
Q

Why does sepsis cause jaundice?

A

Increases haemolysis

Slows down liver function

33
Q

Causes of haemolysis causing jaundice in the neonate

A 
ABO incompatibility 
- excessive haemolysis 
Rheus disease
- excessive haemolysis 
Bruising / cephallhaematoma from birth trauma
- excess of bilirubin production 
Red cell membrane defects e.g. spherocytosis
- excessive haemolysis 
Red cell enzyme defects e.g. G6PD
- excesshaemolysis
34
Q

What causes abnormal conjugation of bilirubin?

A

Gilberts Disease

Criler-Najjar syndrome

35
Q

What is kernicterus a complication of?

A

Neonatal unconjugated jaundice

36
Q

Pathology of kernicterus

A

Unconjugated bilirubin is fat soluble and so can cross the BBB
Neurotoxic and deposits in brain
- especially basal ganglia

37
Q

Presentation of kernicterus

A 
Early signs - encephalopathy 
- poor feeding
- lethargy 
- seizures 
Late consequences
- severe choreathetoid CP
- LDs
- sensorineural deafness
38
Q

Which type of bilirubin can cross the BBB?

A

Unconjugated

39
Q

Treatment of neonatal jaundice

A

Phototherapy

40
Q

What type of jaundice does phototherapy treat?

A

Unconjugated

41
Q

What does phototherapy do?

A

Visible light (NOT UV) converts unconjugated bilirubin to water soluble isomer (photoisomerization)

42
Q

What is exchange transfusion?

A

When phototherapy sometimes doesn’t bring it down efficiently, and have to do this more excessively, then may need a blood transfusion past this threshold

43
Q

What type of jaundice always requires further investigations?

A

Prolonged

44
Q

Causes of unconjugated prolonged neonatal jaundice

A

Hypothyroidism

Breast milk jaundice

45
Q

Causes of conjugated prolonged neonatal jaundice

A 
Anatomical (biliary obstruction) 
- Biliary atresia
- choledochal cyst 
- alagille syndrome 
Neonatal hepatitis 
- viral hepatitis
- parental nutrition 
- glycogen storage disorders
- haemochromatosis  
- urea cycle defects
46
Q

Presentation of biliary atresia causing neonatal jaundice

A

Conjugated jaundice

Pale stools

47
Q

Investigations for biliary atresia

A

Split bilirubin
Stool colour
USS
Liver biopsy

48
Q

What is a choledochal cyst?

A

Developmental condition where there is a cystic abnormality in bile ducts so get obstructive picture

49
Q

What kind of jaundice is a cholechondral cyst?

A

Conjugated jaundice

50
Q

What does a cholechondral cyst present with?

A

Pale stools

Jaundice

51
Q

Investigations of a cholechondral cyst

A

Split bilirubin
Stool colour
USS

52
Q

Pathology of Alagille syndrome

A

Congenital damage to intrahepatic bile ducts

53
Q

Presentation of Alagille Syndrome

A

Intrahepatic cholestasis
Dysmorphism
Congenital heart disease

54
Q

Investigations of alagille syndrome

A

Dysmorphism

Genotype

55
Q

What type of jaundice in infants is ALWAYS abnormal?

A

Conjugated

56
Q

What is the most important test in prolonged jaundice?

A

Split bilirubin

57
Q

What should always be done along with split bilirubin as an investigation for prolonged jaundice in infants?

A

Pale stools In babies

58
Q

What is biliary atresia?

A

Congenital fibro-inflammatory disease of bile ducts leading to destruction of extra-hepatic bile ducts
- so there is no flow of bile into small intestine out of the liver

59
Q

Presentation of biliary atresia

A

Prolonged, conjugated jaundice
Pale stools
Dark urine

60
Q

What can biliary atresia progress to if not identified and treated?

A

Liver failure

61
Q

What is the most common indication for liver transplantation in children?

A

Biliary atresia

62
Q

Treatment of biliary atresia

A

Kasai Portoenterostomy

Paeds (28 decks)

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