Paediatric jaundice Flashcards

1
Q

When dealing with paediatric jaundice - you must appreciate the age of the baby as this can indicate what the cause of jaundice might be - and also how serious it is

What are causes of early infant jaundice - in a baby less than a day old?

A

Jaundice in baby < day is always pathological

Can be due to:

Haemolysis

Sepsis

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2
Q

When dealing with paediatric jaundice - you must appreciate the age of the baby as this can indicate what the cause of jaundice might be - and also how serious it is

What are the causes of intermediate infant jaundice - in a baby 1 day - 2 weeks old?

A

Can be physiological jaundice (ie not pathological)

Can be due to:

  • Breast milk jaundice
  • Haemolysis
  • Sepsis
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3
Q

When dealing with paediatric jaundice - you must appreciate the age of the baby as this can indicate what the cause of jaundice might be - and also how serious it is

What are the main causes of prolonged infant jaundice - in a baby > 2 weeks old?

A

Extrahepatic obstruction

Neonatal hepatitis

Hypothyroidism

Breast milk jaundice

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4
Q

What is meant by physiological jaundice?

A

Jaundice that most babies get to some degree - due to a number of things:

  • Short RBC lifespan (due to HbF)
  • Relative polycythaemia
  • Immature liver function (doesnt conjugate v well yet)

This leads to unconjugated jaundice - that is fairly normal but doesnt happen until after 1 day of life

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5
Q

Compare breatsfeeding & formula feeding in the context of jaundice

A

Breastfeeding typically causes more jaundice and prolongation of jaundice in babies

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6
Q

What are the clinical aspects of breastmilk jaundice?

A

Failry normal - nobody really knows what causes it

Unconjugated jaundice

Can persist up to 12 weeks

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7
Q

If jaundice is normal in babies and most often due to harmless physiological causes - then why do we assess babies with jaundice?

A

Kernicterus - a complication of neonatal jaundice (even physiological i think)

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8
Q

What is kernicterus?

A

Kernicterus = bilirubin-induced brain dysfunction

This happens due to high levels of unconjugated bilirubin crossing the blood brain barrier

Bilirubin is neurotoxic and deposits in the brain - especially in the basal ganglia

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9
Q

Why is it only unconjugated bilirubin that causes kernicterus?

A

Unconjugated bilirubin is water insoluble - and thus fat soluble

This means it can cross the blood-brain barrier

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10
Q

What are the clinical features of kernicterus?

What are the potential consequences of it?

A

Encephalopathy:

  • poor feeding
  • lethargy
  • then seizures

Consequences:

  • severe choreoathetoid cerebral palsy
  • learning difficulties
  • sensorineural deafness
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11
Q

What treatment options are available for treatment of unconjugated jaundice of a neonate?

What are the indications for each?

A

Photo-therapy

Visible light (450nm) causes photoisomerisation of unconjugated bilirubin into a water soluble isomer

This is used when rising unconjugated bilirubin levels are detected - to prevent them getting to a level where kernicterus is a risk

Exchange transfusion

This is the treatment for already very high levels of unconjugated bilirubin - ie just before or at the level causing kernicterus

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12
Q

What are the other causes of early/intermediate unconjugated jaundice in newborns?

A

Sepsis

Haemolysis:

  • ABO or Rhesus incompatible
  • Bruising / cephalhaematoma
  • Red cell membrane defects - eg Spherocytosis
  • Red cell enzyme defects - eg G6PD deficiency

Abnormal conjugation:

  • Gilbert’s disease - common but mild
  • Crigler-Najar syndrome - rare and severe
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13
Q

How would you investigate the following causes of unconjugated jaundice…

a) Sepsis?

Hameolysis caused by:

  • b) ABO or Rhesus incompatibility?
  • c) Bruising / cephalhaematoma?
  • d) Red cell membrane defects - eg spherocytosis?
  • e) “ enzyme defects - eg G6PD deficiency?
A

a) Sepsis - urine & blood cultures, TORCH screen

Haemolysis caused by:

b) ABO / Rhesus - blood group, Direct Coombs Test (DCT)
c) Bruising / cephalhaematoma - examination
d) Spherocytosis etc - blood film
e) G6PD deficiency etc - G6PD assay or similar

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14
Q

Define prolonged infant jaundice

A

Any jaundice of the infant lasting beyond 2 weeks of life

(or 3 weeks for preterm)

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15
Q

What are the main causes of prolonged infant jaundice?

What type of jaundice is seen in each

A

Anatomical (biliary jaundice) - conjugated

Neonatal hepatitis - conjugated

Hypothyroidism - unconjugated

Breast milk jaundice - unconjugated

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16
Q

What is always worrying - conjugated or unconjugated prolonged infant jaundice?

A

Conjugated prolonged infant jaundice is always pathological - thus always requires further investigation

(that doesnt mean that there all causes of unconjugated prolonged infant jaundice are not pathological and not serious)

17
Q

What bloods investigation is most important in prolonged infant jaundice?

What else is it important to examine/investigate?

A

Split bilirubin

Examine stool colour

18
Q

What are the anatomical causes of prolonged infant jaundice?

What are their main features?

A

Biliary atresia (mc)*see bottom:

  • Conjugated jaundice
  • Pale, fatty stools

Choledochal cyst - (cystic dilation of bile ducts):

  • Conjugated jaundice
  • Pale, fatty stools

Alagille syndrome:

  • Intrahepatic cholestasis, dysmorphism, congenital cardiac disease

*Biliary atresia is a serious cause - and should be the first thing you rule out before considering others (incl hepatitis)

19
Q

What is biliary atresia?

What are its mean clinical features?

A

Congenital fibro-inflammatory disease of bile ducts leading to destruction of extra-hepatic bile ducts

Prolonged, conjugated infant jaundice

Really pale stools & dark urine

Progression to liver failure - if not treated

Mc cause of liver transplant in infants

20
Q

What is the treatment for biliary atresia?

A

Kasai portoenterostomy

Must be done < 60 days

(if progressed to bad liver failure - then transplant i assume)

21
Q

What are the anatomical causes of prolonged infant jaundice?

For each, describe very briefly the order of investigation needed for diagnosis

A

Biliary atresia:

  • split bilirubin, stool colour, ultrasound, liver biopsy

Choledochal cyst:

  • split bilirubin, stool colour, ultrasound

Alagille syndrome:

  • dysmorphism, genotyping
22
Q

Neonatal hepatitis is one of the main causes of prolonged infant jaundice

What are the many causes of neonatal hepatitis?

What test diagnoses each?

Horrible card

A

Alpha-1-antitrypsin deficiency - phenotype/level

Galactosaemia - GAL-1-PUT

Tyrosinaemia - amino acid profile

Urea cycle defects - ammonia

Haemochromatosis - iron studies, liver biopsy

Glycogen storage disorders - biopsy

Hypothyroidism - TFTs

Viral hepatitis - serology, PCR

Parenteral nutrition - history

23
Q
A