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Paediatrics > Approach to the Yellow Baby > Flashcards

Approach to the Yellow Baby Flashcards

1
Q

What is included in LFTs?

A

LIVER DAMAGE TESTS

Bilirubin

ALT/AST (alanine aminotransferase/aspartate aminotransferase)

Alkaline phosphatase

Gamma glutamyl transferase (GGT)

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2
Q

What bilirubin measurements are taken?

A

Total bilirubin

“Split” bilirubin – Direct (conjugated) + Indirect (unconjugated)

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3
Q

When is ALT/AST elevated?

A

Elevated in hepatocellular damage (“hepatitis”)

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4
Q

When are alkaline phosphatase and gamma glutamyl transferase (GGT) elevated?

A

Biliary disease

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5
Q

How might liver FUNCTION be tested?

A

Coagulation

Albumin

Bilirubin

(Blood glucose)

(Ammonia)

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6
Q

What measurements are used to assess coagulation?

A

Prothrombin time (PT)/INR

APTT (activated partial thromboplastin time)

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7
Q

How does paediatric liver disease clinically manifest?

A

JAUNDICE

Incidental finding of abnormal blood test

Symptoms/signs of chronic liver disease

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8
Q

Where is jaundice most obvious?

A

Sclera

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9
Q

When does jaundice become visible?

A

Total bilirubin >40-50 umol/l

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10
Q

What is diagnosis of infant jaundice dependent on?

A

Understanding bilirubin metabolism

Age of the infant

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11
Q

Where in bilirubin metabolism does pre-hepatic jaundice occur?

A

Post-mature erythrocytes –> Unconjugated bilirubin

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12
Q

Where does intrahepatic jaundice occur?

A

Unconjugated –> conjugated bilirubin (at liver)

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13
Q

Where does post-hepatic jaundice occur?

A

CHOLESTASIS

Conjugated bilirubin combined with bile in the small intestine to form urobilirubin

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14
Q

What are the classifications of infant jaundice?

A

Early (<24 hours old)

Intermediate (24hrs – 2 weeks)

Prolonged (>2 weeks)

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15
Q

What are the causes of early infant jaundice?

A

ALWAYS PATHOLOGICAL

Haemolysis

Sepsis

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16
Q

What are the causes of intermediate infant jaundice?

A

Physiological, Breast milk, Sepsis, Haemolysis

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17
Q

What are the causes of prolonged infant jaundice?

A

Extrahepatic obstruction, Neonatal hepatitis, Hypothyroidism, Breast milk

18
Q

What are the causes of prolonged infant jaundice?

A

Extrahepatic obstruction, Neonatal hepatitis, Hypothyroidism, Breast milk

19
Q

What is physiological jaundice?

A

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

Relative polycythaemia

Relative immaturity of liver function

Unconjugated jaundice

Develops after first day of life

20
Q

What is breast milk jaundice?

A

Exact reason for prolongation of jaundice in breastfed infants unclear

  • Inhibition of UDP by progesterone metabolite?
  • Increased enterohepatic circulation?

Unconjugated jaundice

Can persist up to 12 weeks

21
Q

What is the cause of kernicterus?

A

Unconjugatedbilirubin is fat-soluble (water insoluble) so can cross blood-brain barrier and become neurotoxic, leaving deposits in the brain

22
Q

What are the early signs of kericterus?

A

Encephalopathy – poor feeding, lethargy, seizures

23
Q

What are the late consequences of kericterus?

A

Severe choreoathetoidcerebral palsy

Learning difficulties

Sensorineural deafness

24
Q

What is phototherapy?

A
  • Treatment for unconjugated jaundice
  • Visible light (450nm wavelength) (not UV) converts bilirubin to water soluble isomer (photoisomerisation)
  • Threshold for phototherapy in infants guided by charts
25
Q

What are some other causes or early/intermediate unconjugated infant jaundice?

A

Sepsis

Haemolysis

Abnormal conjugation

26
Q

What tests do you do to confirn jaundice sepsis?

A

Urine + blood cultures, TORCH screen

27
Q

What are the causes of haemolysis jaundice and how is each tested for?

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

DCT - direct Coombs test

28
Q

What are the causes of “abnormal conjugaton” jaundice and how are they tested for?

A
  • Gilbert’s disease (genotype/phenotype)
  • Crigler-Najjarsyndrome (genotype/phenotype)
29
Q

What is the minimum prolonged jaundice duration for pre-term infants?

A

3 weeks

30
Q

Of the 4 types of prolonged infant jaunidce, which are conjugated and which are unconjugated?

A

Conjugated:

  • Anatomical (biliary obstruction)
  • Neonatal hepatitis

Unconjugated:

  • Hypothyroidism
  • Breast-milk jaundice
31
Q

What are the 3 key messages for prolonged infant jaundice?

A
  1. Conjugated jaundice in infants is always abnormal and always requires further investigation
    • The most important test in prolonged jaundice is a “split” bilirubin
  2. Always assess stool colour in infants with prolonged jaundice
  3. Assessment of prolonged infant jaundice is primarily targeted at diagnosing patients with biliary atresia early
32
Q

What are the 3 causes of bilary obstruction in infants?

A
  • Biliary atresia
  • Choledochal cyst
  • Alagille syndrome
33
Q

How does biliary atresia and choledochal cysts present?

A

Conjugated jaundice

Pale stools

34
Q

How does Alagille syndrome present?

A

•Intrahepatic cholestasis, dysmorphism, congenital cardiac disease

35
Q

What is biliary atresia?

A
  • Congenital fibro-inflammatory disease of bile ducts leading to destruction of extra-hepatic bile ducts
  • Presents with prolonged, conjugated jaundice
  • Pale stools, dark urine
  • Progression to liver failure if not identified and treated
  • Timely diagnosis critical as time to treatment determines prognosis
  • Most common indication for liver transplantation in children
36
Q

What is Kasai portoenterostomy?

A

Biliary atresia treatment

Success rate diminishes rapidly with age

Best results if performed before 60 days (<9 weeks)

37
Q

How is biliary atresia tetsed for?

A

Split bilirubin

Stool colour

Ultrasound

Liver biopsy

38
Q

How are coledochal cysts and and Alagille syndrome tested for?

A
  • Choledochal cyst - split bilirubin, stool colour, ultrasound)
  • Alagille syndrome - dysmorphism, genotype
39
Q

What are the causes of neonatal hepatitis (conjugated jaundice) and now are they tested for?

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)
40
Q
A
41
Q

Summary

A
  • Always ask about stool colour
  • Most important test is a split bilirubin to differentiate unconjugated from conjugated jaundice
  • Conjugated jaundice always requires further investigation and should be considered biliary atresia until proven otherwise

Paediatrics (37 decks)

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