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Exam - M - Paediatrics (Megan) > Jaundice r > Flashcards

Jaundice r Flashcards

1
Q

how may liver disease present in children

A 
jaundice 
growth failure/weight loss
ascites
peripheral neuropathy 
splenomegaly 
muscle wasting 
clubbing 
varices
spider naevi 
petechiae 
peripheral neuropathy
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2
Q

what is the best way to assess liver function

A

coagulation tests

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3
Q

how can you examine jaundice in children

A

visible from >40-50umol/l
check sclera
press on sternum and look for yellow blanching

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4
Q

what other condition causes yellowing of skin

A

beta-carotenaemia

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5
Q

early jaundice

A

<24 hrs old

always pathological

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6
Q

early jaundice causes

A

sepsis

haemolysis

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7
Q

intermediate jaundice

A

24hrs-2wks

common

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8
Q

causes of intermediate jaundice

A

physiological
breast milk
could be haemolysis or sepsis

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9
Q

prolonged jaundice

A

> 2 weeks
3 weeks if preterm
likely to be pathological

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10
Q

causes of prolonged jaundice

A

extra-hepatic obstruction
neonatal hepatitis
hypothyroidism

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11
Q

common causes of haemolysis

A 
ABO incompatibility 
rhesus 
RBC membrane defects e.g. spherocytosis 
traumatic delivery with bruising 
red cell enzyme defects e.g. G6PD
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12
Q

features of pre-hepatic jaundice

A

problem is before the liver
raised levels of unconjugated bilirubin
more bilirubin is being made - could be from haemolysis

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13
Q

features of intrahepatic jaundice

A

problem in in the liver
mixture of conjugated and unconjugated bilirubin
liver is not conjugating well or not excreting well

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14
Q

post-hepatic jaundice features

A

problem is with bile getting out of the liver

elevated conjugated bilirubin

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15
Q

what contributes to physiological jaundice

A

infants have a shorter RBC life span 80-90 days because they have foetal Hb not adult Hb
infants are polycythaemic when born (have high RBC count)
immature liver function

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16
Q

what is breast milk jaundice

A

infants who are breast fed are more likely to become jaundiced
can persist for 12 weeks
always unconjugated
cause not known

17
Q

what is kernicterus

A

jaundice with unconjugated bilirubin that results in long term complications

18
Q

pathophysiology of kernicterus

A

unconjugated bilirubin is fat soluble so can cross the blood brain barrier
it deposits in the brain - particularly at basal ganglia
it is neurotoxic

19
Q

early signs of kernicterus

A

encephalopathy
poor feeding
lethargy
seizures

20
Q

long term complications of kernicterus

A

severe cerebral palsy
learning difficulties
sensorineural deafness

21
Q

investigations for jaundice in newborns

A

urine tests and blood cultures to exclude sepsis
blood group test to exclude rhesus and ABO incompatibility
blood film for RBC defects
genetic testing in severe cases

22
Q

management of unconjugated jaundice in newborns

A

phototherapy

exchange transfusion - this is a more aggressive option, only do it if needed

23
Q

causes of prolonged infant jaundice

A

biliary obstruction - biliary stresia, choledochal cyst, alagille syndrome
hepatitis
hypothyroidism

24
Q

causes of hepatitis in infants

A

A1A deficiency
viral hepatitis
urea cycle defects
glycogen storage disorders

25
Q

is prolonged infant jaundice unconjugated or conjugated?

A

can be either

conjugated jaundice in infants is always abnormal

26
Q

investigations for prolonged infant jaundice

A 
split bilirubin - most important
assess stool colour 
US of liver 
liver biopsy 
TFTs 
genetics
27
Q

what is biliary atresia

A

congenital fibro-inflammatory disease of bile ducts

28
Q

how does biliary atresia prevent bile flow

A

inflammation and destruction of bile ducts causes fibrosis and scarring
this narrows the ducts and prevents flow of bile - cholestasis

29
Q

presentation of biliary atresia

A

conjugated jaundice
pale stools
dark urine

30
Q

prognosis of biliary atresia

A

without early intervention it will lead to progressive liver failure

31
Q

management of biliary atresia

A

kasai portoenterostomy ideally within 60 days

likely to need liver transplant in later life

Exam - M - Paediatrics (Megan) (57 decks)

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