Neonates

Question 1

Add in case of long line infection

Answer 1

Vancomycin

Question 2

Best antibiotic for Listeria infection

Answer 2

Ampicillin

Question 3

Causes platelet sequestration and high output cardiac failure secondary to AV shunting

Answer 3

Kasabach-Merritt

Question 4

First line anticonvulsant for neonates

Answer 4

Phenobarbital

Question 5

Causes of conjugated newborn jaundice

Answer 5

Hepatitis
Congenital infection
Biliary atresia
Cystic fibrosis
Metabolic conditions
Alpha 1 antitrypsin deficiency
TPN
Haemochromatosis

Question 6

Presents early with cyanosis
Absence/hypoplasia of mitral valve and aortic root
Small LV and LA
Large RV and RA

Answer 6

Hypoplastic left heart syndrome

Question 7

Presents in late childhood/teenage years with fatigue or palpitations
R to L shunt with cyanosis and polycythaemia
Characteristic multiple clicks in the left sternal border

Answer 7

Ebstein’s anomaly

Question 8

Presents with hypotonia, bilateral talipes equinovarus and difficulty in sucking and swallowing; may have facial weakness and triangular shaped open mouth
Mothers may have facial weakness or grip myotonia

Answer 8

Congenital myotonic dystrophy

Question 9

How many conditions does the bloodspot screening cover?

Answer 9

Nine

Question 10

Precursor hormones to 11-beta-hydroxylase?

Answer 10

11-deoxycortisol and deoxycorticosterone

Question 11

More common in Japanese

Insidious onset of tachypnoea, cyanosis, increasing respiratory distress, overextended chest, bronchi, coughing

Answer 11

Wilson-Mikity syndrome

Question 12

Threshold of conjugated bilirubin to notify liver team

Answer 12

25

Question 13

Features of foetal alcohol syndrome

Answer 13

IUGR
Microcephaly
Short palpebral fissures
Ocular abnormalities
Joint abnormalities

Question 14

Presents in first few days of life with respiratory alkalosis (usually transient), then high ammonia with normal pH

Answer 14

Urea cycle defect

Question 15

Commonest urea cycle defect

Answer 15

Ornithine transcarbamylase deficiency

Question 16

Clinical features of high ammonia?

Answer 16

Poor feeding
Vomiting
Lethargy
Seizures
Decreased consciousness

Question 17

Smooth philtrum
Smooth upper lip
Microcephaly
Maxillary hypoplasia
Mid face hypoplasia

Answer 17

Foetal alcohol syndrome

Question 18

What kind of sepsis are galactosaemia patients more at risk of?

Answer 18

E coli

Question 19

Presents with murmur, cataracts and hepatosplenomegaly

Answer 19

Rubella