paeds

Question 1

polyhydramnios, low birth weight , non bilious vomiting, epigastric distension, double bubble sign

Answer 1

annular pancreas

Question 2

seizure + port wine stain + intraparenchymal calcifications

Answer 2

sturge weber syndrome

Question 3

widespread mucocutaneous oedema (without pruritus or urticaria) especially in response to stress or trauma (particularly dental procedures)

Answer 3

hereditary angiodema (due to C1 inhibitor deficiency leading to excess levels of bradykinin)

Question 4

common affected areas in hereditary angioedema

Answer 4

affects the skin or mucosal surfaces of upper respiratory or gastrointestinal tract

Question 5

egosyntonic vs egodystonic

Answer 5

egosyntonic - others are distressed by their behaviour
egodystonic - the person themself is distressed from their own behavious

Question 6

Lambert eaton myasthenic syndrome pathophysiology

Answer 6

autoantibody-impaired acetycholine release from presynaptic nerve terminals

Question 7

pansystolic murmur heard loudest in the left lower sternal border

Answer 7

VSD

Question 8

continuous murmur heard in the left infraclavicular area

Answer 8

PDA

Question 9

VSDs are louder the —– the defect is

Answer 9

smaller

Question 10

crescendo-decrescendo harsh systolic murmur best heard at the left upper sternal border

Answer 10

TOF murmur

Question 11

Congenital heart defects that cause Eisemenger syndrome

Answer 11

VSD, ASD, atrioventricular canal defect, PDA. In all these cases, pulmonary pressure increases, leading to reversal of the shunt

Question 12

Childhood seizure disorder characterised by bilateral myoclonic jerks worse in the morning/after sleep deprivation + 1st line treatment?

Answer 12

juvenile myoclonic epilepsy. 1st line tx is valproate

Question 13

4 to 6 Hz irregular polyspike activity on EEG is characteristic of…?

Answer 13

JME

Question 14

fever, bilateral nonexudative conjunctivitis, cervical lymphadenopathy, erythema of the palm/soles, perineal erythema +/- desquamation followed by a macular/mobiliform/target-like rash

Answer 14

kawasaki disease

Question 15

important screening in kawasaki disease

Answer 15

ECHO - coronary artery aneurysm may develop within a few weeks of the onset of disease

Question 16

when is methacholine bronchoprovocation testing indicated?

Answer 16

questioning of asthma dx, developemnt of occupation or iritant0based asthma

Question 17

bochdalek hernia

Answer 17

posterolateral defect in the diaphragm which allows abdominal contents to enter the thorax - 90% of cases are left-sided

Question 18

kartagener’s syndrome

Answer 18

chracterised by primary ciliary dyskinesia and situs inversus