PBR

Question 1

PKU tx is low phenylalanine diet. They tend to need ____ supplementation

Answer 1

tyrosine

Question 2

Expected length of babies:

1 year old should be ___x birth length
4 yr old should be ___x birth length
13 yr old ____x birth length

Answer 2

1 year old should be 1.5x birth length
4 yr old should be 2x birth length
13 yr old 3x birth length

Question 3

single vessel cord, think about what syndrome?

Answer 3

VATER or VACTERL

Question 4

Relative contraindications to DTaP include brief seizure, high fever like > 105, or shock like state. If any of these are greater than ____ out, you still give the vaccine!

Answer 4

4 days

[if within 3 days, take out pertussis component]

Question 5

Waiters tip, grasp intact, can see partial diaphragmatic paralysis

Answer 5

Erb palsy

Question 6

choanal atresia, bilateral syndactyly, cleft palate, craniosynostosis. what syndrome?

A. smith lemli opitz
B. crouzon
C. pierre robin
D. Apert syndrome

Answer 6

D. Apert

(autosomal dominant – key: if BILATERAL syndactyly + craniosynostosis –> slam Apert and move on)

Question 7

4 mo baby with AM irritability, BG 46, labs show lactic acid elevated, AG 16, ammonia normal.

A. glycogen storage type I
B. glycogen storage II pompe
C. Niemann pick
D. isovaleric acidemia

Answer 7

A glycogen storage type I – inability to use glycogen. get fasting hypoglycemia. Give starch.

Question 8

Which is more risky – chorionic villus sampling or amniocentesis?

Answer 8

CVS

Question 9

LBW vs VLBW vs ELBW

Answer 9

LBW <2500 g
VLBW < 1500 g
ELBW <1000 g

Question 10

Supravalvular stenosis – think what syndrome?

Answer 10

Williams

Question 11

baby with hx oligohydramnios on prenatal US, no testes palpable in scrotum, no urine yet, soft abdomen with midline abdominal mass

A. CAH
B. prune belly
C. wilms
D. kallman

Answer 11

B. prune belly

Question 12

Study claims there was a significant difference, but there wasn’t one. What type of error?

Answer 12

type I

Question 13

prader willi is a loss of _____ imprinting on chr15
angelman syndrome is loss of _____ imprinting on chr15

Answer 13

paternal; maternal

Question 14

Expectations for AFP, estriol, Hcg, inhibin on quad screen when worried about T21

Answer 14

AFP – low
estriol – low
Hcg – high
inhibin – high

Question 15

mid parental height equation

Answer 15

female: ([mom height + dad height] - 5 inches)/2
boys: ([mom height + dad height] + 5 inches)/2

can be +/- 2-3 inches

Question 16

In high risk neonates (35-37 weeks with risk factors like G6PD, asphyxia, temp instability, sepsis, acidosis, albumin <3, Rh disease, sibling w/ hx phototherapy, exclusive BF not going well, ABO incompatibility, bruising, cephalohematoma), start phototherapy if total bilirubin is greater than ______ at 24 hrs, greater than _____ at 48 hrs, or greater than ______ at 72 hours.

In medium risk neonates (35-37 weeks without risk factors or >38 weeks with risk factors), total bilirubin threshold increases by ______ compared to above.

In low risk neonates (>38 weeks and well), total bilirubin threshold increases by ______ compared to high risk group.

Answer 16

8 at 24 hrs
11 at 48 hrs
13 at 72 hrs

medium risk threshold increases by 2 (10, 13, 15 respectively)

low risk threshold increases by 4 (12, 15, 17-18 respectively)

Note that none of the above thresholds apply to neonates born <35 weeks!

Question 17

Neonate cord catheters should be placed to what levels?

Answer 17

L3-5
T6-10

Question 18

LGA vs SGA

Answer 18

LGA >3900 g

SGA <2500 g

(50th percentile = 3.25 kg)

Question 19

When do you screen for DDH with imaging?

Answer 19

after 2 weeks of age (2 weeks to 4-6 months you do US, >4-6 months you do XR)