PAEDS

Question 1

Name 5 clinical signs associated with a Patent Ductus arteriosus

Answer 1

Pansystolic machine like murmur
Collapsing Pulse
heaving apex beat
Left subclavicular thrill
wide pulse pressure

Question 2

What is the Management of PDA?

Answer 2

Indomethacin (inhibits prostaglandin) closes

Prostaglandin e1 keeps open for surgery

Question 3

Name common features of turners syndrome

Answer 3

X or XO (deletion of 1 chromosome or short arm of 1 chromosome)

• short stature
• shield chest
• webbed neck
• high arched palate
• cystic hygroma (pre natal diagnosis)
• short fourth metacarpal
• multiple pigmented naevi
• neonatal lymphoedema
• primary amennorhoea (increased gonadotrophins)
• hypothyroidism plus crohns
• bicuspid aortic valve
• coarctation of the aorta

Question 4

Name some common features of Edward’s syndrome

Answer 4

Trisomy 18

• Micrognathia (undersized jaw)
• Low set ears
• Rocker bottom feet
• overlapping of fingers

Question 5

Name Some features of William’s Syndrome?

Answer 5

• Short stature
• learning difficulties
• friendly extrovert personality
• transient neonatal hypercalcaemia
• supravalvular aortic stenosis

Question 6

Name some features of Patau syndrome

Answer 6

Trisomy 13

• microcephalic, small eyes
• cleft lip/palate
• polydactyly
• scalp lesions

Question 7

Name some features of fragile X syndrome

Answer 7

• Learning difficulties
• Macrocephaly
• Long face
• Large ears
• Macro-orchidism
• mitral valve prolapse
• autism

Question 8

What are the features of tetrology of fallot?

Answer 8

Most common cyanotic CHD (except at birth where Transposition of the great arteries is more common). Presents 1-2 months post partum. Right to left shunt. Boot shaped heart on X ray. ECG shows RVH
Features include
- overriding aorta
- pulmonary valve stenosis (ejection systolic murmur)
- right ventricular hypertrophy (often due to the outflow obstruction)
- VSD

Question 9

Describe Noonan syndrome

Answer 9

• Webbed neck
• Pectus excavatum
• short stature
• PULMONARY STENOSIS

Question 10

Describe Pierre Robin Syndrome

Answer 10

• Micrognathia
• posterior deplacement of the tongue that can result in upper airyway obstruction
• cleft palate

NB if family history think Treacher collins syndrome

Question 11

Describe Prader willi syndrome

Answer 11

hypotonia
hypogonadism
obesity

Question 12

What is eisenmengers syndrome?

Answer 12

reversal of a left to right shunt commonly seen in VSD, ASD or more rarely PDA. This is due to the increasing pressures in the right ventrical leading to increased RVH and pulmonary resistance to the point that the shunt reverses.
At this point the patient can become more breathless as the non oxygenated blood in the right ventricle skips the lungs to enter the left ventricle

Question 13

how would you detect intusseption

Answer 13

uss abdo

Question 14

When and how would you treat whooping cough?

Answer 14

Azithromycin or clarithromycin if <21 days since onset of cough and >1
<1 clarithromycin
pregnent women erythromycin