Paeds Yellow Baby Flashcards
name 5 liver functions
Factory - production of proteins - albumin and clotting factors
Breakfast - metabolism of food
Storage centre. - glycogen
Excretion of toxic stuff
Name 2 Bilirubin tests
Total bilirubin
Split bilirubin
What is “Split bilirubin” ?
Direct (conjugated) + indirect (unconjugated)
ALT/AST (alanine aminotransferase/aspartate aminotransferase) are elevated in…
hepatocellular damage (“hepatitis”)
Alkaline phosphate and Gamma glutamyl transferase (GGT) are elevated in…
biliary disease
Name some Liver “function” tests (LFTs)
Bilirubin (total and split)
ALT/AST
Alkaline phosphate
Gamma glutamyl transferase (GGT)
Name some tests to assess liver FUNCTION
Coagulation
Albumin
Bilirubin
Name 2 tests that asses liver function with regards to coagulation
Prothrombin time (PT)/ INR aPTT
What does INR stand for and what is it?
International normalised ratio
measurement of how long it takes for blood to clot
What is the main clinical manifestation of paediatric liver disease?
JAUNDICE
What are other clinical manifestations of paediatric liver disease?
incidental finding of abnormal blood test
symtoms/signs of chronic liver disease
Signs of Chronic Liver Disease in Children
Encephalopathy Jaundice think in terms of liver function: coagulation: - epistaxis -bruising and petechiae think in terms of portal hypertension: varices hepatorenal failure Ascites Think in terms of Cholestasis: fat malabsorption Deficiency of fat-soluble vitamins pruritus play stools dark urine
Other than the usual signs of chronic liver disease common to booth adults and children
what else would you expect to find in kids?
Growth failure
FTT
Jaundice is
yellow discolouration of skin and tissues due to accumulation of bilirubin
Jaundice is most obvious in the…
sclera
jaundice is usually visible when the total bilirubin is >
40-50 mol/L
The diagnosis of infant jaundice is dependent on 2 things….
understanding bilrubin metabolism
The age of infant
When RBCs are broken down
Globin is broken down to amino acids, iron is rested and have I broken down to ….
Biliverdin -> unconjugated bilirubin
Uncojugated bilirubin is water
insoluble
Uncojugated bilirubin is bound to … and transported to the …
albumin
liver
The liver …. uncojugated bilirubin
conjugates
conjugated bilirubin is water
soluble
conjugated bilirubin goes into the
bile
bile goes into the
small intestine
bile is broken down into
urobiligen
what 3 things can happen to urobiligen?
excreted by kidneys
excreted in stool
goes back to liver
If something goes wrong with bilirubin metabolism during the RBC to uncojugated bilirubin stage this is called ….. jaundice
Pre-hepatic jaundice
in Pre-hepatic jaundice there is mostly ….. bilirubin
uncojugated bilirubin
If something goes wrong when the bilirubin is getting conjugated in the liver this is called ….. jaundice
intrahepatic jaundice
In intrahepatic jaundice there is …. bilirubin
mixed bilirubin
uncojugated/ conjugated bilirubin
If something goes wrong from the bile getting to the small intestine this is called
post hepatic jaundice (cholestasis)
Post hepatic jaundice is mostly ….. bilirubin
conjugated
Within how many hours old is early neonatal jaundice classified as
<24hrs
Early neonatal jaundice is always…
pathological
Causes of early neonatal jaundice include…
Haemolysis + Sepsis
Time frame of intermediate neonatal jaundice
(24hrs - 2 weeks)
Causes of intermediate neonatal jaundice include…
physiological, breast milk, sepsis, heaolysis
time frame of prolonged neonatal jaundice
> 2 weeks
Causes of prolonged neonatal jaundice include
Extrahepatic obstruction, neonatal hepatitis, hypothyroidism, breast milk
Physiological jaundice happens because…
RBCs have a …. life span in infants
there is relative …cythaemia
and relative…. of liver function
Shorter
polycythaemia
immaturity
In physiological jaundice there is …. bilirubin
unconjugated
What type of milk is associated with intermediate jaundice of neonates?
Breast milk
-reason unclear
What type of bilirubin is present in breast-milk jaundice?
Unconjugated
In breast-milk jaundice you get more worried and investigate other things more when ….
it last for longer and longer
What is a complication of pre-hepatic (unconjugated bilirubin) jaundice?
Kernicterus
why can unconjugated bilirubin cross the blood-brain barrier in Kernicterus?
because it is fat-soluble (water insoluble)
unconjugated bilirubin is neuro….. and leaves…. in the brain
toxic
deposits
Early signs of kernicterus
encephalopathy- poor feeding, lethargy, seizures
Late consequences of kernicterus
severe choreoathetoid cerebral palsy, learning difficulties, sensorineural deafness
Treatment for unconjugated jaundice
Phototherapy
Phototherapy is
visible light
The light in phototherapy converts bilirubin to
a water soluble isomer
Other causes of unconjugated early/ intermediate infant jaundice
Sepsis
Haemolysis
Abnormal conjugation (congenital stuff)
Haemolytic causes of unconjugated infant jaundice
ABO incompatibility Rhesus disease Bruising/cephalhaematoma Red cell membrane defects (e.g. spherocytosis) Red cell enzyme defects (e.g. G6PD)
Conjugated causes for prolonged infant jaundice
Anatomical (biliary obstruction)
Neonatal hepatitis
Unconjugated causes for prolonged infant jaundice
Hypothyroidism
Breast-milk jaundice
Conjugated jaundice in infants is always… and …. requires further investigation
abnormal and always
the most important test in prolonged jaundice is a
split bilirubin
Causes of prolonged jaundice - biliary obstruction
BILLIARY ATRESIA
Choledochal cyst
Alagille syndrome
Always asses the stool … in infants with prolonged jaundice
colour
Biliary atresia is
Congenital fibro-inflammatory disease of bile ducts leading to destruction of extra-hepatic bile ducts
Biliary atresia presents with
prolonged, conjugated jaundice
In biliary atresia stools are.. and urine is …
pale and dark
In biliary atresia there can be progression to … if not identified and treated
liver failure
primary treatment for biliary atresia
Surgical fix - Kasai
duodenum connected up to liver
Assessment of prolonged infant jaundice is primarily targeted at diagnosing patients with…
biliary atresia early
What investigation do you ultimately need in biliary atresia?
liver biopsy
Causes of neonatal hepatitis
Alpha-1- antitrypsin deficiency
galctosaemia
