Jaundice r Flashcards
how may liver disease present in children
jaundice growth failure/weight loss ascites peripheral neuropathy splenomegaly muscle wasting clubbing varices spider naevi petechiae peripheral neuropathy
what is the best way to assess liver function
coagulation tests
how can you examine jaundice in children
visible from >40-50umol/l
check sclera
press on sternum and look for yellow blanching
what other condition causes yellowing of skin
beta-carotenaemia
early jaundice
<24 hrs old
always pathological
early jaundice causes
sepsis
haemolysis
intermediate jaundice
24hrs-2wks
common
causes of intermediate jaundice
physiological
breast milk
could be haemolysis or sepsis
prolonged jaundice
> 2 weeks
3 weeks if preterm
likely to be pathological
causes of prolonged jaundice
extra-hepatic obstruction
neonatal hepatitis
hypothyroidism
common causes of haemolysis
ABO incompatibility rhesus RBC membrane defects e.g. spherocytosis traumatic delivery with bruising red cell enzyme defects e.g. G6PD
features of pre-hepatic jaundice
problem is before the liver
raised levels of unconjugated bilirubin
more bilirubin is being made - could be from haemolysis
features of intrahepatic jaundice
problem in in the liver
mixture of conjugated and unconjugated bilirubin
liver is not conjugating well or not excreting well
post-hepatic jaundice features
problem is with bile getting out of the liver
elevated conjugated bilirubin
what contributes to physiological jaundice
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
what is breast milk jaundice
infants who are breast fed are more likely to become jaundiced
can persist for 12 weeks
always unconjugated
cause not known
what is kernicterus
jaundice with unconjugated bilirubin that results in long term complications
pathophysiology of kernicterus
unconjugated bilirubin is fat soluble so can cross the blood brain barrier
it deposits in the brain - particularly at basal ganglia
it is neurotoxic
early signs of kernicterus
encephalopathy
poor feeding
lethargy
seizures
long term complications of kernicterus
severe cerebral palsy
learning difficulties
sensorineural deafness
investigations for jaundice in newborns
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
management of unconjugated jaundice in newborns
phototherapy
exchange transfusion - this is a more aggressive option, only do it if needed
causes of prolonged infant jaundice
biliary obstruction - biliary stresia, choledochal cyst, alagille syndrome
hepatitis
hypothyroidism
causes of hepatitis in infants
A1A deficiency
viral hepatitis
urea cycle defects
glycogen storage disorders
is prolonged infant jaundice unconjugated or conjugated?
can be either
conjugated jaundice in infants is always abnormal
investigations for prolonged infant jaundice
split bilirubin - most important assess stool colour US of liver liver biopsy TFTs genetics
what is biliary atresia
congenital fibro-inflammatory disease of bile ducts
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
presentation of biliary atresia
conjugated jaundice
pale stools
dark urine
prognosis of biliary atresia
without early intervention it will lead to progressive liver failure
management of biliary atresia
kasai portoenterostomy ideally within 60 days
likely to need liver transplant in later life
