Jaundice r Flashcards

(31 cards)

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
is prolonged infant jaundice unconjugated or conjugated?
can be either | conjugated jaundice in infants is always abnormal
26
investigations for prolonged infant jaundice
``` split bilirubin - most important assess stool colour US of liver liver biopsy TFTs genetics ```
27
what is biliary atresia
congenital fibro-inflammatory disease of bile ducts
28
how does biliary atresia prevent bile flow
inflammation and destruction of bile ducts causes fibrosis and scarring this narrows the ducts and prevents flow of bile - cholestasis
29
presentation of biliary atresia
conjugated jaundice pale stools dark urine
30
prognosis of biliary atresia
without early intervention it will lead to progressive liver failure
31
management of biliary atresia
kasai portoenterostomy ideally within 60 days | likely to need liver transplant in later life