Jaundice (Megan)

Question 1

what tests are best when assessing liver function?

Answer 1

INR (prothrombin time) and albumin levels.

Question 2

What is the most common presentation of liver disease in children?

Answer 2

Jaundice

Question 3

What do ALT and AST levels show?

Answer 3

These are elevated if you have damaged your heaptocytes

Question 4

What do AP and GGT levels show?

Answer 4

These are elevated if you have a problem with your bile duct

Question 5

What is the pathway for bilirubin metabolism?

Answer 5

• RBCs are broken down by the ER system.
• the haem part is broken down into biliverdin, this is converted into unconjugated bilirubin by biliverdin reductase.
• unconjugated bilirubin is bound to albumin and is taken to the liver where it is conjugated.
• its then excreted into the bile duct and into the intestines.
• in the small intestine its converted into urobilinogen, then in the gut its converted into stercobilin which gives stool its dark colour.
• some of the urobilinogen is excreted via the kidneys into the urine, and some is reabsorbed back through the liver to repeat the cycle.

Question 6

what enzyme converts biliverdin into unconjugated bilirubin?

Answer 6

Biliverdin reductase.

Question 7

How is unconjugated bilirubin conjugated?

Answer 7

with gluculuronic acid

Question 8

At what ages is jaundice pathological?

Answer 8

<24 hrs

> 2 weeks (usually)

Question 9

what is physiological jaundice?

Answer 9

Usually when the HbF has a shorter lifespan (80-90) and they are destroyed sooner and the immature liver can’t conjugate the bilirubin quick enough leading to unconjugated-bilirubin jaundice.

This doesn’t occur until AFTER the 1st day of life.

Question 10

how long can breast milk jaundice occur for?

Answer 10

Up to 12 weeks of life (BUT if it begins after 2 weeks, or 3 weeks in pre term babies it should still be investigated).

Question 11

who is allowed a longer threshold for jaundice?

Answer 11

babies who are breast fed usually have jaundice for longer than formula fed babies.

Term babies = allow 2 weeks before investigations.
Pre-term babies = allow 3 weeks before investigations.

Question 11

who is allowed a longer threshold for jaundice?

Answer 11

babies who are breast fed usually have jaundice for longer than formula fed babies.

Term babies = allow 2 weeks before investigations.
Pre-term babies = allow 3 weeks before investigations.

Question 12

what is a severe complication of increased levels of unconjugated bilirubin?

Answer 12

Kernicterus - the unconjugated bilirubin is fat soluble so can cross the blood brain barrier and deposit in the basal ganglia.

Question 13

What are the effects of kernicterus?

Answer 13

Poor feeding
seizures
Cerebral palsy
Encephalopathy

Question 14

what is a common therapy for unconjugated bilirubin?

Answer 14

Photo- therapy: it converts unconjugated bilirubin into water soluble so it can be excreted through the liver.

Question 15

what are the main causes unconjugated jaundice?

Answer 15

physiological
breast milk
sepsis
haemolysis
hypothyroidism

Question 16

what are the main causes of conjugated jaundice?

Answer 16

Biliary atresia
alpha 1 antitrypsen
viral hepatitis

Question 17

what type of jaundice are early jaundices?

Answer 17

Mostly unconjugated

Question 18

what type of jaundice is prolonged jaundice?

Answer 18

can be either conjugated or unconjugated

Question 19

when is jaundice classified as prolonged?

Answer 19

> 2 weeks in term infants.

> 3 weeks in pre term infants.

Question 20

what type of jaundice in babies is always abnormal?

Answer 20

Conjugated jaundice e- this suggests obstructive jaundice.

Question 21

what type of jaundice in babies is always abnormal?

Answer 21

Conjugated jaundice e- this suggests obstructive jaundice.

Question 22

what signs are always seen for obstructive jaundice?

Answer 22

Pale stools

dark urine

Question 23

What is biliary atresia?

Answer 23

A congenital fibre-inflammatory disease of the bile ducts which starts distally then works its way proximally into the hepatic system.

Without treatment it leads to liver failure and death.

Question 24

what does biliary atresia lead to?

Answer 24

increased levels of conjugated bilirubin.

Most common cause of liver transplant.

Question 25

why is the urine dark in obstructive jaundice?

Answer 25

the conjugated bilirubin is still water soluble so can still be excreted through the kidneys and theres more of it

Question 26

what investigations can be done for biliary atresia?

Answer 26

Split bilirubin
stool colour
ultrasound
liver biopsy

Question 27

what is the treatment for biliary atresia?

Answer 27

Kasai Portoenterostomy (palliative) and usually winds up in liver transplant:

the gut is brought up to the liver and the bile is excreted direly into the bowel, with the stomach attaching to the bile further down.

Question 28

what are the signs of biliary atresia?

Answer 28

pale stools
dark urine
symptoms persisting > 2 weeks
yellow skin

Question 29

What is cholestasis?

Answer 29

decrease in bile flow due to impaired obstruction of hepatocytes or obstruction of bile through intra or extra hepatic ducts.