2020 PREP

Question 1

What lab and imaging surveillance is required for patients with Beckwith-Wiedemann syndrome?

Answer 1

• Abdominal US every 3 months until 8 years of age
• Alpha-fetoprotein every 2-3 months until 4 years of age

Question 2

What finding on blood smear is pathognomonic for babesiosis?

Answer 2

Maltese cross

Question 3

What is the most common cause of congenital hypothyroidism?

Answer 3

Thyroid dysgenesis

Question 4

What is the confirmatory testing for narcolepsy?

Answer 4

Polysomnography with multiple sleep latency testing

Question 5

What is Lemierre syndrome?

Answer 5

Septic thrombophlebitis of internal jugular vein in association with head and neck infection

Question 6

When should vaccinations be given after HSCT?

Answer 6

At least 6 months after HSCT and stopping immunosuppressants

Question 7

What are the most common manifestations of Stickler syndrome?

Answer 7

Vision and hearing abnormalities

Question 8

What are the 3 components of Pierre Robin sequence?

Answer 8

Micrognathia, cleft palate, and glossoptosis

Question 9

Which patients should receive Tamiflu treatment on an outpatient basis?

Answer 9

Children younger than 5 (especially younger than 2) with confirmed OR suspected flu infection (even without rapid antigen testing)

Question 10

What is the mechanism of injury in epidural hematoma?

Answer 10

Rupture of middle meningeal artery

Question 11

Which disorder is associated with acrodermatitis enteropathica?

Answer 11

Zinc deficiency

Question 12

What random glucose level is diagnostic of DM?

Answer 12

> 200

Question 13

What HbA1c is diagnostic of DM?

Answer 13

≥ 6.5%

Question 14

What is the main symptomatic treatment for myasthenia gravis and what is its mechanism of action?

Answer 14

Pyridostigmine (AChE inhibitor)

Question 15

What is the function of the MIBG scan?

Answer 15

Evaluates for pheochromocytoma and neuroblastoma

Question 16

Which skin disorder is pictured here?

Answer 16

Icthyosis vulgaris

Question 17

What is the treatment for icthyosis vulgaris?

Answer 17

Topical emollient contaning alpha-hydroxy acid

Question 18

What are some signs and symptoms of severe C. diff?

Answer 18

Fever, leukocytosis > 15,000, and AKI

Question 19

When is universal screening for lead levels?

Answer 19

1 and 2 years of age

Question 20

At what lead level should treatment for iron-deficiency anemia be considered?

Answer 20

> 5

Question 21

How is neonatal chronic hepatitis C diagnosed?

Answer 21

Hepatitis C antibodies at 18 months of age (checking beforehand may reflect transplacental maternal antibodies)

Question 21

How is neonatal chronic hepatitis C diagnosed?

Answer 21

Hepatitis C antibodies at 18 months of age (checking beforehand may reflect transplacental maternal antibodies)

Question 22

What degree of curvature on scoliometer warrants XR?

Answer 22

> 7 degrees

Question 23

What age group is most susceptible to Kingella septic arthritis and osteomyelitis?

Answer 23

< 4 years of age

Question 24

How long after thelarche do girls typically have their first menstrual cycle?

Answer 24

2-2.5 years

Question 25

What is the definition of delayed puberty in girls?

Answer 25

No thelarche by 13 years of age OR no menarche by 16 years of age in a girl with normal breast development

Question 26

What is the first-line treatment for Guillain-Barre syndrome?

Answer 26

IVIG

Question 27

What is the CSF hallmark of Guillain-Barre syndrome?

Answer 27

Albuminocytologic dissociation (high protein with normal cell count)