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Paediatrics > The Yellow Baby > Flashcards

The Yellow Baby Flashcards

1
Q

What are the LFTs?

A

Bilirubin - total/split
ALT/AST ratio
Alkaline phosphatase
GGT

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2
Q

What is ALT/AST ratio a marker of?

A

Hepatic damage

>2 in alcoholic liver disease

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3
Q

What is alkaline phosphatase a marker of?

A

Raised in biliary disease but also raised in pregnancy and diseases where there is increase osteoblast activity

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4
Q

What is GGT a marker of?

A

Biliary disease but also alcoholic liver disease

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5
Q

To actually assess liver function what tests are better?

A

Coagulation - PT/INR/APTT
Albumin
Bilirubin
Blood glucose & ammonia sometimes

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6
Q

What does PT measure?

A

Vit K dependent clotting factors - 1, 2, 5, 7, 10

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7
Q

What does APTT measure?

A

Other clotting factors

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8
Q

What does high albumin suggest?

A

Dehyration

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9
Q

What does low albumin suggest?

A

Protein loss or liver dysfunction

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10
Q

How can liver disease manifest in children?

A

Signs/symptoms
Jaundice
Incidental abnormal blood test

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11
Q

What are the signs and symptoms of liver disease?

A

Oestrogen related ones: spider naevi, liver palms etc.
Portal HTN, varices, splenomegaly, hypersplenism, ascites
Ammonia related encephalopathy
Hepatorenal failure
Malabsorption, rickets
Clotting factor related ones: petechiae/bruising/epistaxis
Peripheral neuropathy, hypotonia

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12
Q

Define jaundice

A

Yellow discolouration of skin and other tissues due to accumulation of bilirubin

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13
Q

At what total bilirubin level does jaundice usually become visible

A

> 40-50umol/l

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14
Q

Where is jaundice most visible?

A

Sclera

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15
Q

Describe the bilirubin metabolism pathway

A

Post-mature erythrocytes broken down by RES into haem
Haem broken down into biliverdin
Biliverdin broken down into unconjugated bilirubin by biliverdin reductase
UC bilirubin travels to liver attached to albumin
Liver conjugates bilirubin with UDP glucuronyl transferase
Conjugated bilirubin transformed into bile and then urobilinogen in the small intestine
Can be reuptaken by enterohepatic circulation or excreted by kidneys/gut
In gut becomes stercobilin (responsible for colour of shit)

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16
Q

How is neonatal jaundice classified?

A

Age
Early (<24h) - always pathological
Immediate (24h-2wks)
Prolonged (>2wk)

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17
Q

What are the causes for early jaundice?

A

Sepsis, haemolysis

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18
Q

What are the causes for immediate jaundice?

A

Sepsis, haemolysis, physiological jaundice, breast milk jaundice

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19
Q

What are the causes for prolonged jaundice?

A

Extrahepatic obstruction, neonatal hepatitis, breast milk jaundice, hypothyroidism

20
Q

What is physiological jaundice?

A

RBCs have shorter lifespan in infants (80-90d) so relative polycythaemia & relative immaturity of liver function –> unconjugated jaundice

21
Q

What is the mechanism behind breast milk jaundice?

A

We don’t entirely know - perhaps inhibition of UDP by progesterone metabolite or increased enterohepatic circulation –> unconjugated jaundice

22
Q

How long can breast milk jaundice last for?

A

Up to 12 weeks

23
Q

What are causes for haemolysis in early/immediate unconjugated infant jaundice?

A 
ABO incompatibility
Rh disease
Bruising/cephalhaematoma
Red cell membrane defect, e.g. spherocytosis
Red cell enzyme defect, e.g. G6PD
24
Q

What two other conditions can cause immediate/early unconjugated infant jaundice?

A

Gilbert’s disease - common & mild

Crigler-Najjar syndrome - v. rare & serious (UDP deficiency)

25
Q

What tests would you do if you suspected sepsis?

A

Blood culture/urine culture

ToRCH screen

26
Q

What tests would you do if you suspected ABO incomptability/Rh disease?

A

Blood group, DCT

27
Q

What tests would you do if you suspected gilbert’s/crigler-Najjar syndrome?

A

Genotype/phenotype

28
Q

What is kernicterus?

A

Unconjugated bilirubin is fat soluble can cross BBB and accumulate in brain
It is neurotoxic

29
Q

What are the early signs of kernicterus?

A

Lethargy, seizures, poor feeding (encephalopathy)

30
Q

What are the late signs of kernicterus?

A

Severe choreoatheoid cerebral palsy, learning difficulties, sensorineural deafness

31
Q

How do you treat unconjugated jaundice?

A

Phototherapy - visible light at 450nm changes bilirubin into water soluble isomer

May need exchange transfusion if more premature/higher level of bilirubin

32
Q

Define prolonged infant jaundice

A

Jaundice lasting more than 2 weeks (or 3 weeks in prems)

33
Q

What can cause prolonged infant jaundice?

A

Anatomical (biliary tree obstruction)
Neonatal hepatitis
Hypothyroidism
Breast milk jaundice

34
Q

Conjugated jaundice is always what?

A

Abnormal and requires further Ix

35
Q

What are the biliary obstructions that can cause conjugated prolonged jaundice?

A

Biliary atresia - CJ, pale stools
Choledochal cysts - CJ, pale stools
Alagille syndrome - intrahepatic cholestasis, facial dysmorphism, congenital cardiac dx

36
Q

What is biliary atresia?

A

Congenital fibro-inflammatory disease of bile ducts leading to destruction of extra-hepatic bile ducts

37
Q

What is the presentation of biliary atresia?

A

Pale stools, CJ, dark urine

Can progress to liver failure if not Ix and Rx

38
Q

How do you Rx biliary atresia?

A

Kasai portoenterostomy (within 60 days for best results)

39
Q

What investigations would you do for biliary atresia?

A

Split bilirubin, stool colour, USS, liver biopsy

40
Q

What Ix would you do for choledochal cyst?

A

Split bilirubin, stool colour, USS

41
Q

What Ix do you do for Alagille syndrome?

A

Genotyping

42
Q

What are other rarer causes of prolonged infant jaundice?

A 
Alpha-1-antitryspin deficiency 
Galactosaemia
Tyrosinaemia 
Urea cycle defects
Haemachromatosis
Glycogen storage disorders
Hypothyroidism
Viral hepatitis
Parenteral nutrition
43
Q

What test do you do for galactosaemia?

A

GAL-1-PUT

44
Q

What test do you do for trysoniaemia?

A

Amino acid profile

45
Q

What test do you do for urea cycle defects?

A

Ammonia

46
Q

What tests do you do for haemochromatosis?

A

Iron studies, liver biopsy

47
Q

What tests do you do for glycogen storage disorders?

A

Biopsy

Paediatrics (35 decks)

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