III

Question 1

Jervell and Lange-Nielsen syndrome

Answer 1

autosomal recessive

family Hx of sudden death, congenital sensorineural deafness and QT interval ~600

Question 2

patients with long QT are at high risk for what

Answer 2

syncope, life threatening ventricular arrhythmias, and sudden death

Question 3

Tx inherited QT syndrome

Answer 3

beta blocker like propanolol

pacemaker if symptomatic

Question 4

ethosuximide is first line for what

Answer 4

absences seizures

Question 5

beta blocker to avoid in long qt

Answer 5

sotalol

Question 6

electrolyte derangements that can cause long QT

Answer 6

hypoCa
hypo K
hypoMg

Question 7

medications that can cause long QT

Answer 7

macrolide, fluoroquinolone, psychotropic, opioids, antiemetics, anti arrhythmics

Question 8

autosomal dominant form of long QT

Answer 8

romano-ward syndrome

Question 9

poor eater with dry eyes, photophobia, dry scaly skin, follicular hyperkeratosis

Answer 9

Vitamin A deficiency

Question 10

dry silver gray plaques on bulbar conjunctiva

bitot spots

Answer 10

vitamin a deficiency

Question 11

dry vs wet beri beri

Answer 11

dry- symmetrical peripheral neuropathy with sensory and motor deficits
wet- cardiomegaly, cardiomyopathy, CHF, peripheral edema, tachycardia

Question 12

ascorbic acid deficiency

Answer 12

impaired collagen synthesis and damaged CT

ecchymoses, petechiae, bleeding gums, hyperkeratosis, Sjogrens

Question 13

symptoms hypervitaminosis A

Answer 13

anorexia, pruritis, lack of weight gain, increased irritability, limitation of motion, tender swelling of the bones, alopecia, seborrheic cutaneous lesions , increased intracranial pressure, hepatomegaly

Question 14

murmur in TOF

Answer 14

harsh, systolic ejection murmur at left upper sternal border

Question 15

boot shaped heart

Answer 15

TOF

Question 16

signs of a “tet-spell”

Answer 16

knee-chest positioning

inhaled oxygen

Question 17

4 anomalies in TOF

Answer 17

right ventricular outflow tract obstruction
right ventricular hypertrophy
overriding aorta
VSD

Question 18

internal carotid artery dissection in child

Answer 18

after trauma to soft palate with a sharp object

Question 19

which enzyme is decreased in asian newborns

Answer 19

uridine diphosphoglconurate glucoronosyltransferase

Question 20

when is exchange transfusion indicated for jaundice

Answer 20

total bili >20-25

Question 21

initial Sx of necrotizing enterocolitis

Answer 21

hypothermia and lethargy

vomiting, abdominal distention

Question 22

pneumatosis intestinalis

Answer 22

air in bowel showing double line or train track appearance

Question 23

double bubble

Answer 23

duodenal atresia

Question 24

failure to pass meconium within 48 hour

Answer 24

hirchsprung disease

Question 25

type of hydrocephalus with severe neonatal hemorrhage

Answer 25

obstructive

Question 26

pneumatosis intestinalis common in what neonates

Answer 26

premature

Question 27

pyloric stenosis most common in

Answer 27

first born boys typically 3-5 weeks with projectile nonbilious vomiting
palpable olive shaped mass

Question 28

immediate care for pyloric stenosis

Answer 28

hydration and K replacement

Question 29

predisposing factor for volvulus

Answer 29

malrotation of midgut

Question 30

bilious vomiting

Answer 30

midgut volvulus

Question 31

fastest and most accurate method for diagnosing malrotation of midgut

Answer 31

upper GI series, barium swallow

Question 32

finding of ligament of treitz on right side of abdomen

Answer 32

malrotation- corkscrew pattern

Question 33

Ladd procedure

Answer 33

fixing the bowel in non-rotated position to minmize recurrent volvulus risk

Question 34

clinical features of biliary atresia

Answer 34

well appearing
development of jaundice, pale stools or dark urine, hepatomegaly, conj hyperbilirubinemia, mild elevation of transaminases

Question 35

Dx biliary atresia methods

Answer 35

US
hepatobiliary scintigraphy
liver biopsy
intraoperative cholangiogram

Question 36

Tx biliary atresia

Answer 36

hepatoportoenterostomy

liver transplant

Question 37

future procedure for biliary atresia

Answer 37

kasai procedure

Question 38

crigler-najjar and gilberts are deficiencies in what

Answer 38

UDP glucuronyl transferase

Question 39

type of hyperbilirubinemia in alloimmune hemolytic disease

Answer 39

unconjugated

Question 40

type of hyperbilirubinemia in biliary atresia

Answer 40

conjugated

Question 41

which hyperbilirubinemia is always pathogenic

Answer 41

conjugated

Question 42

theory of gastroenteritis and intussusception

Answer 42

inflammation of peyers patches leading to a lead point.

Question 43

currant jelly stools

Answer 43

intussusception

Question 44

imaging to Dx intussusception

Answer 44

US

Question 45

target sign

Answer 45

intussusception

Question 46

cyanosis aggravated by feeding and relieved by crying

Answer 46

choanal atresia

Question 47

CHARGE syndrome

Answer 47

coloboma. heart defects, atresia of choanae, renal anomalies, growth impairment and ear abnormalities/deafness

Question 48

dx imaging for choanal atresia

Answer 48

CT

Question 49

absolute contraindication to breastfeeding

Answer 49

galactosemia

Question 50

treatment for constipation in toddelr

Answer 50

increase dietary fiber
limit cows milk intake
laxative
enemas

Question 51

risk factors for infantile iron deficient anemia

Answer 51

prematurity, maternal iron deficiency, cows milk before 12 months