PEDS

Question 1

What is stage IV-S neuroblastoma?

Answer 1

Children <1 yr old with specific presentation of localized primary bone marrow involvement and metastatic spread to liver an dskin

Question 2

What is Sandifer’s ?

Answer 2

Realtes to GERd; consists of a twisting of the neck (potentially assoc with failure to thrive)

Question 3

Spontaneous neonatal perforation causing free air is most likely coming from?

Answer 3

Greater curvature of stomach. Other causes of sudden distension secondary to intestinal perforation ar eless likely to be associated with significant itnraperitoneal hemorrhage.

Question 4

How do we confirm diagnosis of ToF

Answer 4

Echocardiography

Question 5

How do you manage cyanotic spells a/w ToF

Answer 5

Supplemental O2
Sedation with morphine
Beta blocker

Question 6

What is a Blalock Taussig shunt

Answer 6

For a palliative increase in pulmonary blood flow, you can use this shutn to connect subclavian artery to the pulmonary artery

Question 7

most common indication for operative repair of ToF

Answer 7

Increasing cyanosis and cyanotic spells

Question 8

How do we close PDA?

Answer 8

Indomethacin by means of cyclo-oxygenase inhibition, which decreases production fo endogenous prostaglandins

Question 9

How do we keep PDA open?

Answer 9

Prostaglandin infusion

Question 10

Von Hippel Lindau

Answer 10

Associated with renal cell CA, renal cysts, CNS tumors, pheo

Question 11

Li Fraumeni syndrome

Answer 11

defect in p53 – > childhood sarcomas, breast CA, brain tumors, leukemia, adrenal CA

Question 12

Beckwith Weidemann

Answer 12

Nephroblastoma (Wilms tumor), macroglossia, visceromegaly, hyperinsulinemci hypoglycemia

Question 13

McCune Albright

Answer 13

Bone deformities, cafe au lait, precocious puberty