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The Yellow Baby Flashcards

(47 cards)

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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?

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A

Extrahepatic obstruction, neonatal hepatitis, breast milk jaundice, hypothyroidism

20
Q

What is physiological jaundice?

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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?

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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?

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A

Up to 12 weeks

23
Q

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

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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?

Study These Flashcards
A

Gilbert’s disease - common & mild

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

25
What tests would you do if you suspected sepsis?
Blood culture/urine culture | ToRCH screen
26
What tests would you do if you suspected ABO incomptability/Rh disease?
Blood group, DCT
27
What tests would you do if you suspected gilbert's/crigler-Najjar syndrome?
Genotype/phenotype
28
What is kernicterus?
Unconjugated bilirubin is fat soluble can cross BBB and accumulate in brain It is neurotoxic
29
What are the early signs of kernicterus?
Lethargy, seizures, poor feeding (encephalopathy)
30
What are the late signs of kernicterus?
Severe choreoatheoid cerebral palsy, learning difficulties, sensorineural deafness
31
How do you treat unconjugated jaundice?
Phototherapy - visible light at 450nm changes bilirubin into water soluble isomer May need exchange transfusion if more premature/higher level of bilirubin
32
Define prolonged infant jaundice
Jaundice lasting more than 2 weeks (or 3 weeks in prems)
33
What can cause prolonged infant jaundice?
Anatomical (biliary tree obstruction) Neonatal hepatitis Hypothyroidism Breast milk jaundice
34
Conjugated jaundice is always what?
Abnormal and requires further Ix
35
What are the biliary obstructions that can cause conjugated prolonged jaundice?
Biliary atresia - CJ, pale stools Choledochal cysts - CJ, pale stools Alagille syndrome - intrahepatic cholestasis, facial dysmorphism, congenital cardiac dx
36
What is biliary atresia?
Congenital fibro-inflammatory disease of bile ducts leading to destruction of extra-hepatic bile ducts
37
What is the presentation of biliary atresia?
Pale stools, CJ, dark urine | Can progress to liver failure if not Ix and Rx
38
How do you Rx biliary atresia?
Kasai portoenterostomy (within 60 days for best results)
39
What investigations would you do for biliary atresia?
Split bilirubin, stool colour, USS, liver biopsy
40
What Ix would you do for choledochal cyst?
Split bilirubin, stool colour, USS
41
What Ix do you do for Alagille syndrome?
Genotyping
42
What are other rarer causes of prolonged infant jaundice?
``` Alpha-1-antitryspin deficiency Galactosaemia Tyrosinaemia Urea cycle defects Haemachromatosis Glycogen storage disorders Hypothyroidism Viral hepatitis Parenteral nutrition ```
43
What test do you do for galactosaemia?
GAL-1-PUT
44
What test do you do for trysoniaemia?
Amino acid profile
45
What test do you do for urea cycle defects?
Ammonia
46
What tests do you do for haemochromatosis?
Iron studies, liver biopsy
47
What tests do you do for glycogen storage disorders?
Biopsy

Paediatrics (35 decks)

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