Jaundice Flashcards
What LFT is raised in heatocellular damage e.g. hepatitis?
ALT/AST
What LFT is raised in biliary diease?
Alkaline phosphatase
Gamma glutamyl transferase (GGT)
How does paediatric liver disease present?
Jaundice
Incidental finding
Rarely with signs of chronic liver disease: epistaxis, encephalopathy, ascities, clubbing, hypotonia, spider navei, brusing, growth faliure
What is jaundice?
Yellow discolouration of skin and tissues due to accumulation of excess bilirubin. Most obvious in sclera
Usually visiable when total bilirubin >40-50 umol/L
Normal in neontaes 1-13umol/L
Normal in children up to 18 1-8 umol
What are the different causes for Jaundice?
Pre-hepatic e.g. increased haemolysis
Intrahepatic- liver diease
Post- hepatic- obstruction stopping bile from getting out e.g. cholestasis
What are the causes of neonatel jaundice (neonate=infant less 4 weeks old)?
<24hrs old always patholgical- haemolysis or sepsis
24hrs-2weeks old can be breast mild, sepsis or haemolysis or physiological
> 2 weeks old - extrahepatic obstruction, neonatal hepatitis, hypothryoidism, breast milk
Why does physiological jaundice occur?
Shorter life span of RBCs in infants, so more turn over.
Relative polycythaemia
Relative immaturity of liver function (unconjugated jaundice)
explain Breast milk Jaundice
We don’t really understand why but babies who are breast fed are more likely to develop jaundice.
Will be unconjugated jaundice and last up to 12 weeks.
Why is it so important babies with jaundice are assessd?
Unconjugated bilirubin is fat-soluable/ water insoluable, so it can cross the BBB and deposit in the brain causing brain damage (this condition is called Kernicterus)
What is Kernicterus and what are the signs?
Unconjagated bilirubin crosses BBB and depositis in brain which can lead to brain damage.
Early signs: encephalopathy, porr feeding, lethargy, seizures
Consquences: cerebal palsy, learning difficultlies, sensorineural deafness
How is jaundice treated to prevent Kernicterus?
For unconjugated jaundice phototherapy 450nm wavelength converts bilirubin in to water soluable isomer.
Or for higher levels:
exchange transfusion, some of babies blood is taken and replaced with bagged blood to reduce bilirubin level.
What bilirubin is raised in pre-hepatic jaundice?
Mostly unconjuagted
What bilirubin is raised in intrahepatic jaundice?
Mixed unconjugated and conjuagted bilirubin
What bilirubin is raised in post-hepatic jaundice?
Mostly conjugated
What are the causes of prolonged infant jaundice and how can they be subdivided?
Biliary obstruction (anatomical issue) and Neonatal hepatitis cause mostly conjugated bilirubin rise. (conjuagted jandice is always abnormal)
Where are hypothryoidism and Breast-milk jaundice mostly cause rise in unconjuagted bilirubin.
What are the potential causes of biliary obstruction and how do they present?
Biliary atresia and choledochal cyst- both cause conjugated jaundice and pale stools.
algille syndrome- intrahepatic cholestasis, dysmorphism, congential cardiac disease
How is biliary atresia treated?
Kasai portoenterostomy (surgery)
Best results if performed before 60 days/9 weeks of life
What investigations are done in prolonger jaundice?
Split bilirubin levels (decides in conjugated or unconjuagted bilirubin responsible)
Stool sample
Ultrasound
Liver biopsy
Genotype (if thinking Alagille syndrome)
