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Paediatrics > Approach to the yellow baby (LIVER) > Flashcards

Approach to the yellow baby (LIVER) Flashcards

1
Q

What are the functions of the liver?

A
  • Produces many of the important enzymes & proteins (clotting factors)
    -Absorption, digestion and metabolism-need bile to help you absorb fat
  • Stores Glc as glycogen, albumin etc
  • Clearance of toxic products-metabolism and excretion
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2
Q

What is included in LFTs?

A
  • Bilirubin (total and split bilirubin (direct (conjugated) +indirect (unconjugated))
  • ALT/AST
  • Alkaline phosphatase
  • GGT
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3
Q

What causes a change in ALT/AST?

A

Elevated in hepatocellular damage (hepatitis)

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4
Q

What LFTs indicate biliary disease?

A

ALP &/or GGT elevated

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4
Q

What LFTs indicate biliary disease?

A

ALP &/or GGT elevated

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5
Q

What rises when there is a problem in liver which stops the flow of bile from the liver?

A

BILIRUBIN

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6
Q

If truly want to test the function of the liver what can be used?

A
  • Coagulation (PT/INR, APTT)
  • Albumin
  • Bilirubin-bile clearance

(Damage - BG (hypoglycaemia), Ammonia(elevated))

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7
Q

What are the clinical manifestations of paediatric liver disease?

A

JAUNDICE

Incidental finding of abnormal blood test

Symptoms/signs of chronic liver disease (same as adults + growth failure)

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8
Q

What is jaundice and when is it usually visible?

A

Yellow discolouration of skin & tissues due to accumulation of bilirubin

Usually most obvious in sclera (distinguishes between true jaundice and beta carotene anaemia)

Usually visible when total bilirubin >40-50umol/l

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9
Q

What is pre-hepatic jaundice?

A

Refers to any cause of jaundice where the problem lies before the liver

MOSTLY UNCONJUGATED

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10
Q

When there is a problem in the liver itself what jaundice can occur?

A

Intrahepatic jaundice

Mixed=unconjugated/conjugated

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11
Q

What is post hepatic jaundice also known as?

A

Cholestasis-obstructive process stopping bile getting out

MOSTLY CONJUGATED

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12
Q

Neonatal jaundice can be classified by age: what are these classifications?

A

Early (<24hrs old) - Always pathological
- Haemolysis, sepsis

Intermediate (24hrs-2 weeks)
- Physiological, breast milk, sepsis, haemolysis

Prolonged (>2 weeks)
- Extrahepatic obstruction, neonatal hepatitis, hypothyroidism, breast milk

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13
Q

Why do get ‘physiological’ jaundice?

A

Shorter RBC life span in infants (80-90 days)

Relative polycythaemia

Relative immaturity of liver function

  • Unconjugated jaundice
  • Develops after 1st day of life
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14
Q

Do we know why breast fed infants jaundice is more likely to last longer than formula fed babies?

A

Exact reason for the prolongation of jaundice in breastfed infants is unclear

Breast fed infants also more likely to get jaundice

  • Unconjugated jaundice
  • Can persist up to 12 weeks
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15
Q

Can kernicterus only happen with high levels of unconjugated jaundice?

A

YESSSSSSS

16
Q

What causes kernicterus and what are the signs of it?

A

Unconjugated bilirubin is fat soluble (water insoluble) so can cross BBB-neurotoxic and deposits in the brain

Early signs=encephalopathy-poor feeding, lethargy, seizures

Late consequences-severe choreoathetoid CP, learning difficulties, sensorineural deafness

17
Q

What is the treatment for unconjugated jaundice?

A

Phototherapy- NOT UV is just visible light - converts bilirubin to water soluble isomer (photoisomerization)

Threshold for phototherapy in infants guided by charts

18
Q

What are some important causes of haemolysis in infants?

A
  • ABO incompatibility
  • Rhesus disease
  • Bruising/cephalhematoma
  • Red cell membrane defects (e.g. spherocytosis)
  • Red cell enzyme defects (e.g. G6PD)
19
Q

What are some important causes of haemolysis in infants?

A
  • ABO incompatibility (Blood group, DCT)
  • Rhesus disease (Blood group, DCT)
  • Bruising/cephalhematoma (clinical exam)
  • Red cell membrane defects (e.g. spherocytosis) (Blood film)
  • Red cell enzyme defects (e.g. G6PD) (G6PD assay)
20
Q

What can cause abnormal conjugation?

A

Gilbert’s disease-common, mild

Crigler-Najjar syndrome-v.rare, severe

(if suspected look at genotype/phenotype)

21
Q

What assessments used to look for sepsis as a cause of unconjugated infant jaundice?

A

Urine + blood cultures, TORCH screen

22
Q

What is prolonged infant jaundice?

A

Jaundice persisting beyond 2 weeks of life (3 weeks for preterm infants)

Causes:
- Anatomical (biliary obstruction)-conjugated
- Neonatal hepatitis-conjugated

  • Hypothyroidism (UNconjugated)
  • Breast milk jaundice (UNconjugated)
23
Q

Conjugated jaundice in infants is … and always requires further testing

A

ALWAYS ABNORMAL

24
Q

What is the most important test in prolonged jaundice?

A

Split bilirubin

25
Q

What can cause biliary obstruction and therefore prolonged jaundice?

A
  • Biliary atresia-conjugated jaundice, pale stools
  • Choledochal cyst-conjugated jaundice, pale stools

ALWAYS assess stool colour in infants with prolonged jaundice

  • Alagille syndrome - intrahepatic cholestasis, dysmorphism, congenital cardiac disease
26
Q

What is biliary atresia and what does it present as?

A

Congenital fibro inflam disease of bile ducts that leads to destruction of extra-hepatic bile ducts

Presents with prolonged, conjugated jaundice

Pale stools, dark urine

Progression to liver failure if not identified or treated - treatment promptly determines prognosis

Most common indication for liver transplantation in children

27
Q

What is the treatment for biliary atresia?

A

Kasai portoenterostomy

  • Success rate diminishes rapidly with age
  • Best results if performed before 60 days (<9weeks)
28
Q

Assessment of prolonged infant jaundice is primarily targeted at what?

A

Diagnosing patients with biliary atresia early

29
Q

Investigations done for the causes of prolonged jaundice -biliary obstruction?

A
  • Split bilirubin
  • Stool culture
  • USS
  • Liver biopsy
  • Dysmorphism
  • Genotype
30
Q

Prolonged especially conjugated jaundice and know they don’t have biliary atresia what should be thought about?

A

NEONATAL HEPATITIS

-A1AT deficiency
- Galactosaemia
- Glycogen storage disorders
- Hypothryoidism

Paediatrics (17 decks)

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