bonus questions

Question 1

Give 3 causes of cyanotic congenital heart disease

Answer 1

• tetralogy of Fallot (TOF)
• transposition of the great arteries (TGA)
• tricuspid atresia

Question 2

What is the initial management for suspected cyanotic congenital heart disease?

Answer 2

Administer prostaglandin E1 (e.g., alprostadil) to maintain a patent ductus arteriosus in ductal-dependent congenital heart defects.

Question 3

What are some causes of a false positive sweat test for cystic fibrosis?

Answer 3

• Malnutrition
• Adrenal insufficiency
• Nephrogenic diabetes insipidus
• Hypothyroidism, hypoparathyroidism
• G6PD deficiency

Question 4

Why is aspirin avoided in children u16 and give an example of an exception

Answer 4

• Risk of reye’s disease: swelling in liver and brain
• kawasaki disease is an exception

Question 5

What is used to treat children with cystic fibrosis who are homozygous for the delta F508 mutation

Answer 5

Lumacaftor/Ivacaftor (Orkambi)
* lumacaftor increases the number of CFTR proteins that are transported to the cell surface
* ivacaftor is a potentiator of CFTR that is already at the cell surface, increasing the probability that the defective channel will be open and allow chloride ions to pass through the channel pore

Question 6

Evidence of bowel sounds in a respiratory exam of a neonate should make you consider what condition

Answer 6

congenital diaphragmatic hernia

Question 7

What condition is indicated by the presence of 6 or more cafe au lait spots

Answer 7

Neurofibromatosis - mainly NF1

Question 8

What dose of adrenaline is given for anaphylaxis in a child <6 months old

Answer 8

100 - 150 micrograms

Question 9

What dose of adrenaline is given for anaphylaxis in a child 6 months - 6 years old

Answer 9

150 micrograms

Question 10

What dose of adrenaline is given for anaphylaxis in a child 6-12 years old

Answer 10

300 micrograms

Question 11

What is the prognosis of minimal change disease

Answer 11

prognosis is good, although relapse is common
2/3 will have later recurrent episodes

Question 11

What investigations should be done in infants younger than 3 months with fever

Answer 11

Full blood count
Blood culture
C-reactive protein
Urine testing for urinary tract infection
Chest radiograph only if respiratory signs are present
Stool culture, if diarrhoea is present