Approach to the yellow baby Flashcards

1
Q

Which enzyme is notably elevated in hepatocellular damage?

A
Alanine aminotransferase (ALT)
Aspartate aminotransferase (AST)
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2
Q

Which enzymes are elevated in biliary disease?

A

Alkaline phosphatase

Gamma glutamyl transferase (GGT)

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3
Q

What are some tests to assess liver function?

A
Coagulation
Albumin
Bilirubin
BG
Ammonia
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4
Q

How do we test coagulation?

A

Prothrombin time

APTT

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5
Q

How might jaundice present?

A

Incidental finding on abnormal blood test

Symptoms/signs of chronic liver disease

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6
Q

What are some signs of chronic liver disease in children?

A
Growth failure
Encephalopathy
Portal hypertension
Varices
Splenomegaly
Ascites
Hypotonia
Clubbing
Hepatorenal failure
Epistaxis
Bruising and petechiae
Muscle wasting
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7
Q

How is chronic liver disease related to Rickets?

A

Rickets may occur secondary to VitD deficiency

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8
Q

Is unconjugated bilirubin water soluble?

A

No

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9
Q

Is conjugated bilirubin water soluble?

A

Yes

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10
Q

How is unconjugated formed?

A

Post-mature erythrocytes broken down to haem
Haem to biliverdin
Biliverdin reductase breaks it down to unconjugated bilirubin

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11
Q

How does bilirubin become conjugated?

A

Binds to albumin to be transported to liver
Glucuronic acid added by glucuronyl transferase
This forms conjugated bilirubin

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12
Q

How is conjugated bilirubin excreted?

A

Excreted through bile into intestine
De-conjugated by β-glucuronidase
Reabsorbed into enterohepatic circulation to be excreted in stool

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13
Q

Where is the problem in pre-hepatic jaundice?

A

Conversion of post-mature erythrocytes to unconjugated bilirubin
Mostly results in unconjugated bilirubin

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14
Q

Where is the problem in intrahepatic jaundice?

A

Conjugation of bilirubin in the liver

Result is mixture of unconjugated and conjugated bilirubin in the liver

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15
Q

Where is the problem in post-hepatic jaundice?

A

Excretion of conjugated bilirubin

Result is mostly conjugated bilirubin

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16
Q

Is jaundice in a neonate always pathological?

A

Yes

17
Q

What are the usual causes of jaundice in a neonate?

A

Haemolysis

Sepsis

18
Q

What are the usual causes for a 0-14 day old baby?

A

Physiological
Breast milk
Sepsis
Haemolysis

19
Q

What are the usual causes of jaundice in a baby >2 weeks?

A

Extrahepatic obstruction
Neonatal hepatitis
Hypothyroidism
Breast milk

20
Q

What kind of bilirubin predominates in physiological jaundice?

A

Unconjugated

21
Q

What is physiological jaundice?

A

Shorter RBC life span in infants (80-90 days)
Relative polycythaemia
Relative immaturity of liver function
Develops after first day of life

22
Q

What are some likely causes of breast milk jaundice?

A

Inhibition of UDP by progesterone metabolite

Increased enterohepatic circulation

23
Q

What kind of bilirubin predominates in breast-milk jaundice?

A

Unconjugated

24
Q

How is kernicterus caused?

A

Unconjugated bilirubin crosses BBB

Neurotoxic and deposits in brain

25
Q

What are some signs of kernicterus?

A

Encephalopathy
Poor feeding
Lethargy
Seizures

26
Q

What are some late consequences of kernicterus?

A

Severe choreoathetoid palsy
Learning difficulties
Sensorineural deafness

27
Q

What is phototherapy used for?

A

Treating unconjugated bilirubin by photoisomerisation

Converts bilirubin to water soluble isomer

28
Q

How do we assess red cell enzyme defects in haemolysis?

A

G6PD assay

29
Q

How do we assess red cell membrane defects in spherocytosis related haemolysis?

A

Blood film

30
Q

What kind of bilirubin results from hypothyroidism related jaundice?

A

Unconjugated

31
Q

What are some causes of biliary obstruction in jaundice?

A

Biliary atresia
Choledochal cyst
Alagille syndrome

32
Q

What are some signs and symptoms of biliary atresia?

A

Prolonged conjugated jaundice
Pale stool
Dark urine

33
Q

How is biliary atresia treated?

A

Kasai portoenterostomy

Most common indication for liver transplant in children

34
Q

What do biliary atresia and choledochal cyst have in common?

A

Conjugated jaundice

Pale stools