QUESTION: What is the diagnosis?
A) Vascular Ring
B) Esophageal Atresia
C) Laryngotracheoesophageal cleft
D) Laryngocele✅ - CORRECT ANSWER(S):
A)
WRONG ANSWERS:
B)
C)
D)
BACKGROUND:
GI = N/V/Ab Pain
Renal = Diabetes Insipidus
Neuro = tremor, ataxia, coma
QUESTION: Most effective tx for this type of poisoning?
A) Activated Charcoal
B) Sodium Bicar
C) Glucagon
D) Hemodialysis✅ - CORRECT ANSWER(S):
D) Hemodialysis bc this is lithium toxicity -- other mgmt includes whole bowel irrigation
w/polyethylene glycol & IV saline rehydration.
WRONG ANSWERS:
A) Used for ______ overdose
B) Used for TCA overdose
C) Used for Bblocker overdose
BACKGROUND:
- 1 wk old infant
- "Clunk" with adducting legs while applying downward and lateral pressure on knees
,QUESTION: What sign is this?
A) Barlow
B) Psoas
C) Ortolani
D) Galeazzi✅ - CORRECT ANSWER(S):
A) Barlow --> DDH
WRONG ANSWERS:
B) Pain elicited by passively extending thigh of pt lying on side --> appendicitis
C) Hip relocation and "clunk" w/abduction of legs and lifting up on trochanters -->DDH
D) Discrepancy in height of flexed thighs (affected limb is shortened) -->DDH
BACKGROUND:
- 1mo Caucasian F
- 3cm pink-red macule on thigh
- Explain to mom, not get bigger, but will darken to a purple color over time
- Mom asks about assoc'd conditions
QUESTION: Which of the following constellation of symptoms is seen in a syndrome that is
also assoc'd w/this birthmark?
A) Axillary freckling, iris hamartomas
B) Posterior fossa malformations, coarc of aorta, and eye abnorm
C) Facial angiofibromas, shagreen patches, subependymal nodules, epilepsy
D) Varicose veins, soft tissue hypertrophy on trunk✅ - CORRECT ANSWER(S):
D) This is a port-wine stain
WRONG ANSWERS:
A) This is NF
B) This is PHACE Syndrome
C) This is Tuberous Sclerosis
BACKGROUND:
- 1wk term male in NICU for aortic stenosis & poor feeding
- Cards waiting until older to correct heart since adequate systemic BP
- PO feeds poor, requiring most feeds via NG
- Labs show consistent hypercalcemia
QUESTION: What diagnosis explains cardiac anomaly + hypercalcemia?
A) Down Syndrome
B) 22q11 deletion
C) Prader Willi
D) Williams Syndrome✅ - CORRECT ANSWER(S):
D) Williams Syndrome
WRONG ANSWERS:
A) No hypercalcemia in Downs
B) DiGeorge --> conotruncal cardiac anomalies, hypoplastic thymus, and HYPOcalcemia
C) No hypercalcemia in PWS;
Neonatal hypotonia --> asphyxia
Feeding diff (poor suck) --> FTT
Weak cry, genital hypoplasia, hypothalamic & pituitary dysfxn
BACKGROUND:
- 2 Groups, each of 100 pts w/cervical cancer
- Group A = New Drug, Q = 96/100 go into remission
- Group B = Std Tx = 80/100 go into remission
QUESTION: What is the odds ratio for improved treatment with Q?
A) 0.1
B) 0.17
C) 0.36
D) 6.0✅ - CORRECT ANSWER(S):
D) Odds Ratio =
(RemissionGroupA x NoRemissionGroupB)
/
(NoRemissionGroupA x RemissionGroupB)
WRONG ANSWERS:
A)
B)
C)
BACKGROUND:
- 2 hr old female in NICU
- FT, 5180g via in vitro
- Omphalocele prev eval'd by surg
- Now jittery w/BG at 12
- PE = macroglossia, facial nevus flammeus, midface hypoplasia, & infraorbital creases
QUESTION: Which of the following is she at risk for?
A) Acute Lymphoid Leukemia
B) Osteosarcoma
C) Medullary Thyroid Cancer
D) Hepatoblastoma✅ - CORRECT ANSWER(S):
, D) This is Beckwith-Wiedemann Syndrome (11p15) --> Wilms and Hepatoblastoma (do US and
measure serum AFP)
WRONG ANSWERS:
A)
B)
C)
BACKGROUND:
- 2 wk old infant
- spontaneous vag deliv at 38 wks
- Exclusively breastfeeds
- Elevated phenylalanine levels
- No PE abnormalities
QUESTION: What is the next step in mgmt?
A) Order plasma amino acids
B) Order assay for dihydropteridine reductase
C) Switch to low-phenylalanine formula
D) Switch to soy formula✅ - CORRECT ANSWER(S):
A) Do this to confirm PKU (def of phenylalanine hydroxylase)
WRONG ANSWERS:
B)
C) Don't do this until confirmed DX
D) This is required for those with galactosemia, no PKU
BACKGROUND:
- 2yo male
- eyes moving rapidly/randomly/erratically in all directions
- yesterday, arms/legs jerk sporadically and involuntarily
- today, difficulty walking
- 2 months ago, had strep pharyngitis treated with 10days of amox
QUESTION: What is the next best step?
A) Measure urinary vanillylmandelic acid
B) Obtain an ASO titer
C) Perform EEG
D) Reassurance✅ - CORRECT ANSWER(S):
A) This is opsoclonus-myoclonus syndrome ("dancing eyes, dancing feet")
-- presents prior to 3yo
-- 40-50% have neuroblastoma, which secretes vanillylmandelic acid (excreted in urine)
-- get a 24-hr measurement of catecholamine levels
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