Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Paediatrics > Hepatology > Flashcards

Hepatology Flashcards

1
Q

What is the defintion of early jaundice?

A

<24 hours old

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What is significant about early jaundice?

A

It is always pathological

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

If a neonate presented with early jaundice, what would be your differential diagnoses?

A
  • Haemolysis
    • ABO incompatibility
    • Rhesus disease
    • Bruising/cephalhaematoma
    • Hereditary spherocytosis
    • G6PD deficiency
  • Congenital infection/Sepsis
  • Metabolic - galactosaemia
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

If you suspected haemolysis as a cause of early jaundice, what investigations would you do?

A
  • Blood grouping
  • G6PD assay
  • Blood film
  • Examination - bruising/cephalohaematoma
  • Coombs test
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

If you suspected sepsis as a cause of early jaundice, what investigations would you do?

A
  • Blood cultures
  • Urine dipstick and culture
  • TORCH screen
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What is the definition of intermediate jaundice?

A

24hrs – 2 weeks

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

If a child presented with jaundice after 1 week, what would your differential diagnoses be?

A
  • Physiological jaundice
  • Breast milk jaundice
  • Sepsis
  • Haemolysis
  • Polycythaemia
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What is physiological jaundice?

A

Jaundice caused by:

  • Low activity of glucuronosyltransferase -> normally converts unconjugated to conjugated bilirubin
  • Shorter life span of fetal red blood cells (80 to 90 days)
  • Relatively low conversion of bilirubin to urobilinogen by the intestinal flora

Develops after first day of life

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

How long can phsyiological jaundice last for?

A

Up to 1 month

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What is breast milk jaundice?

A

Jaundice caused by taking breast milk - Exact reason for prolongation of jaundice in breastfed infants unclear:

  • Inhibition of UDP by progesterone metabolite
  • Increased enterohepatic circulation

Unconjugated jaundice

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What type of bilirubin would you expect to see in physiological jaundice?

A

Unconjugated bilirubin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What type of bilirubin would you expect to see in breast milk jaundice?

A

Unconjugated bilirubin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

How long can breast milk jaundice last for?

A

Up to 12 weeks

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What is prolonged jaundice?

A

>2 weeks

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

How would you assess prolonged jaundice in a child?

A
  • Split bilirubin
  • Stool and urine colour
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

If a neonate presented with jaundice at 3 weeks, and serum bilirubin showed conjugated bilirubin, what would be your differential?

A
  • Bile duct obstruction - biliary atresia, choledochal cyst
  • Neonatal hepatitis syndrome - multiple causes
  • Intrahepatic biliary hypoplasia - Alagille syndrome
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

If a neonate developed jaundice at 3 weeks, and the serum bilirubin was unconjugated, what would you differential diagnosis be?

A
  • Prolonged Breast milk jaundice
  • Hypothyroidism
  • Infection
  • Haemolysis
  • High GI obstruction
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

What is biliary atresia?

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

How does biliary atresia present?

A
  • Faltering growth
  • Pale urine
  • Dark stools
  • Hepato-(+/- spleno-)megaly
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

What is the risk of not treating biliary atresia?

A

Liver failure - time to treatment determines prognosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

How would you investigate for biliary atresia?

A
  • Split bilirubin - conjugated bilirubin
  • Stool colour
  • US - contracted/absent gallbladder
  • Liver Biopsy
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

How would you treat biliary atresia?

A

Kasai portoenterostomy - lopp of the jejunum is anastamosed to the cut surface of the porta hepatis, allowing bile to drain

Best results if performed before 60 days

23
Q

What are Choledocal cysts?

A

Cystic dilatations of the extra-hepatic biliary system

24
Q

How would a child with a choledocal cyst present?

A
  • Abdo pain
  • Palpable mass
  • Cholangitis/jaundice
  • Pale stool/dark urine
25
Q

How would you investigate a suspected choledocal cyst?

A
  • Split bilirubin
  • Stool colour
  • US scan
26
Q

What is Alagille Syndrome?

A

Autosomal dominant condition, with children with traingular faces, skeletal abnormalities, congenital heart disease (PS), renal tubular disorders and intrahepatic biliary hypoplasia

27
Q

If you suspected Alagille syndrome, how would you investigate?

A

Genotyping

28
Q

What are causes of neonatal hepatitis syndrome?

A
  • Alpha-1-antitrypsin deficiency
  • Galactosaemia
  • Tyrosinaemia
  • Urea cycle defects
  • Haemochromatosis
  • Glycogen storage disorders
  • Hypothyroidism
  • Viral hepatitis
29
Q

What can be a complication of jaundice if left untreated for too long?

A

Kernicterus

30
Q

How would you initially approach investigating a child with conjugated hyperbilirubinaemia?

A

Screen for

  • Infection
  • Genetic causes - alpha 1 antitrypsin, CF, galactosaemia
  • Metabolic causes

Abdominal US Scan

31
Q

If a child with conjugated hyperbilirubinaemia had a dilated biliary tree, what would be the suspected diagnosis?

A

Choledocal cyst

32
Q

If a child with conjugated hyperbilirubinaemia had a normal biliary tree on US scan, what would be the next step of investigation?

A

TBIDA radionuclide scan - determines excretion of bile from the tree - whether the bile duct is patent or not

33
Q

What is kernicterus?

A

Encephalopathy resulting from deposition of unconjugated bilirubin in the basal ganglia and brainstem nuclei. Happens when unconjugated bilirubin exceeds albumin binding capacity

34
Q

Why can unconjugated bilirubin cross the blood-brain barrier?

A

It is fat soluble

35
Q

In acute kernicterus, how will the child change at first?

A
  • Decreased alertness
  • Hypotonia
  • Poor feeding
36
Q

In acute kernicterus, how can the child change in the first intial phase of lethargy, poor feeding and hypotonia?

A
  • Irritable
  • Hypertonia of extensor muscles
  • Retrocolis - head drawn back
  • Opisthotonus - arched back
  • Seizures
  • Coma
37
Q

What can develop in the context of chronic kernicterus?

A

Choreoathetoid CP - due to basal ganglia damage

  • Hypotonia, hyperreflexia, delayed acquisition of motor milestones
  • Extrapyramidal – athetosis, chorea, upper limbs > lower limbs
  • High frequency hearing loss
  • Upward gaze
38
Q

What is haemolytic disease of the newborn/ABO incompatibility?

A

Maternal IgG antibodies with specificity for the ABO blood group system pass through the placenta to the fetal circulation where they can cause hemolysis of fetal red blood cells which can lead to fetal anemia and HDN

39
Q

What is Rhesus haemolytic disease?

A

Typically occurs only in some second or subsequent pregnancies of Rh negative women where the fetus’s father is Rh positive, leading to a Rh+ pregnancy. During birth, the mother may be exposed to the infant’s blood, and this causes the development of antibodies, which may affect the health of subsequent Rh+ pregnancies.

40
Q

What test could you do to look for ABO incompatibility?

A

Coomb’s test

41
Q

What is the the incidence of biliary atresia?

A

1/15000

42
Q

How common is conjugated hyperbilirubinaemia in a neonate?

A

Rare

43
Q

What type of jaundice does haemolysis cause?

A

Unconjugated jaundice - overloads albumin carrying capacity and liver processing capacity

44
Q

Why does hepatic and post-hepatic jaundice cause pale stools and dark urine?

A

Bile doesn’t get into the intestine -> no stercobilin to make faeces brown

Conjugated bilirubin is water soluble and accumulates, moving into the blood stream -> carried to kidneys where it can cross glomerulus -> excreted in urine, making it dark

45
Q

How would you treat jaundice?

A
  • Treat the cause
  • Hydrate and feed
  • Determine severity of hyperbilirubinaemia
    • Mild - moderate: Phototherapy
    • Severe: Exchange transfusion
  • Immunoglobulin
46
Q

How do you determine whether to use exchange transfusion or phototherapy?

A
  • Plot bilirubin on gestation specific chart
  • Determine Rate of change
47
Q

What form of therapy would you use in the context of rapidly rising serum bilirubin?

A

Exchange transfusion

48
Q

How would you assess jaundice in a neonate?

A
  • Assess severity
  • Determine gestation
  • Determine age
  • Are they well or unwell
  • Risk factors
  • Needing treatment
49
Q

What type of jaundice can congenital infection cause?

A

CONJUGATED/UNCONJUGATED - depending on cause

50
Q

What type of jaundice is caused by bile duct obstruction (e.g. biliary atresia/choledocal cyst)?

A

CONJUGATED - bile backs up - dark urine pale stool

51
Q

What type of jaundice is caused by neonatal hepatitis syndrome?

A

CONJUGATED

52
Q

What type of jaundice can occur in hypothyroidism?

A

Unconjugated

53
Q

What type of jaundice can occur in high GI obstruction?

A

Unconjugated

54
Q

What are causes of unconjugated prolonged jaundice?

A
  • Prolonged Breast milk jaundice - most common
  • Haemolysis
  • Infection
  • Hypothyroidism
  • High GI obstruction

Paediatrics (27 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions