Paeds Yellow Baby Flashcards by alex brown

name 5 liver functions

Factory - production of proteins - albumin and clotting factors
Breakfast - metabolism of food
Storage centre. - glycogen
Excretion of toxic stuff

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Name 2 Bilirubin tests

Total bilirubin

Split bilirubin

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What is “Split bilirubin” ?

Direct (conjugated) + indirect (unconjugated)

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ALT/AST (alanine aminotransferase/aspartate aminotransferase) are elevated in…

hepatocellular damage (“hepatitis”)

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Alkaline phosphate and Gamma glutamyl transferase (GGT) are elevated in…

biliary disease

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Name some Liver “function” tests (LFTs)

Bilirubin (total and split)
ALT/AST
Alkaline phosphate
Gamma glutamyl transferase (GGT)

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Name some tests to assess liver FUNCTION

Coagulation
Albumin
Bilirubin

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Name 2 tests that asses liver function with regards to coagulation

Prothrombin time (PT)/ INR
aPTT

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What does INR stand for and what is it?

International normalised ratio

measurement of how long it takes for blood to clot

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What is the main clinical manifestation of paediatric liver disease?

JAUNDICE

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What are other clinical manifestations of paediatric liver disease?

incidental finding of abnormal blood test

symtoms/signs of chronic liver disease

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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

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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

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Jaundice is

yellow discolouration of skin and tissues due to accumulation of bilirubin

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Jaundice is most obvious in the…

sclera

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jaundice is usually visible when the total bilirubin is >

40-50 mol/L

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The diagnosis of infant jaundice is dependent on 2 things….

understanding bilrubin metabolism

The age of infant

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When RBCs are broken down

Globin is broken down to amino acids, iron is rested and have I broken down to ….

Biliverdin -> unconjugated bilirubin

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Uncojugated bilirubin is water

insoluble

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Uncojugated bilirubin is bound to … and transported to the …

albumin

liver

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The liver …. uncojugated bilirubin

conjugates

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conjugated bilirubin is water

soluble

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conjugated bilirubin goes into the

bile

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bile goes into the

small intestine

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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