Pediatric Board Review Questions

Question 1

Central cyanosis occurs when

Answer 1

deoxygenated hemoglobin exceeds 3 g/dL

ie Hgb 12, cyanotic if 3 is deoxygenated. So O2 sat would be 9/12, or 75% when you see cyanosis

Question 2

Cause of prolonged QT interval

Answer 2

hypocalcemia
Hypercalcemia conversely causes shortened QT

Question 3

Oxygen delivery to alveoli

Answer 3

(ml/kg/min) = alveolar MV x FiO2
Alveolar MV = TV x RR

Question 4

Increasing CO will change O2 delivery but not content

Answer 4

reminder is all

Question 5

Incidence of Single UA

Answer 5

1%. More common in twins (3-4x)

Question 6

Pre-E vs severe pre-E

Answer 6

Pre-E: SBP>140, DBP > 90
Severe is SBP>160, DBP >110

Question 7

T18 quad screen

Answer 7

low / low / low / normal = T18

Question 8

Teratogenic effects of isotretinoin

Answer 8

TGA, triangular face, microtia, hypertelorism, epicentral folds, small arms, HC > 95%

Question 9

Intrauterine hypoxia favors which organs

Answer 9

heart, brain, adrenals

Question 10

Blood returning from placenta to fetus has PaO2 of

Answer 10

20-30

Question 11

Most oxygenated blood in fetus travels from placenta via UV into IVC and _____ of blood travels through PFO to LA to LV and then to aorta
_____ of descending aorta blood flow goes to placenta by way of UA

Answer 11

1/3; 45%

Question 12

Cyanosis, single heart sound, L superior axis on ECG

Answer 12

Tricuspid atresia

Question 13

Hunter syndrome inheritance

Answer 13

x-linked; Hurler, Sanfillipo, Morquio, Sly are AR

Question 14

Crouzon syndrome

Answer 14

proptosis, maxillary hypoplasia, craniosynostosis

Question 15

Cat-eye syndrome

Answer 15

anal atresia, coloboma of iris, extra part of chromosome 22, TAPVR

Question 16

Meckel Gruber syndrome

Answer 16

Polydactyly, cystic dysplastic kidneys, encephalocele

Question 17

What are essential AA

Answer 17

Methionine, valine, leucine, threonine

Arginine is essential in preterm

Question 18

highest fatty acid concentration in mature BM (which one)

Answer 18

oleic acid

Question 19

Human colostrum compared to mature BM

Answer 19

more protein

Question 20

in CAH, aldosterone is

Answer 20

low

Question 21

Na deficit equation

Answer 21

[na desired - na current][0.6][weight]

Question 22

Profound metabolic acidosis, slightly elevated ammonia

Answer 22

organic acidemia

Question 23

Severely elevated ammonia, respiratory alkalosis

Answer 23

urea cycle

Question 24

Zelwegger syndrome

Answer 24

cerebro-hepato-renal syndrome. AR. Absence of hepatic and renal peroxisomes. pex gene
Hypomyelination, high forehead, hypo plastic supraorbital ridges, HM, chondrodysplasia punctata, eye, renal cysts
Dx: Increased very long chain fatty acids

Question 25

Chronic granulomatous disease and dx test

Answer 25

Chronic granulomatous dz = recurrent infections with catalase + bacteria or fungi Nitroblue tetrazolium test or dihydrorhodamine 123 test

Question 26

SCID presentation

Answer 26

diarrhea, FTT, scaly eruption combined with EOSINOPHILIA

Question 27

Tx for infants born to mothers with gonorrhea

Answer 27

single IV CTX

Question 28

Lactase reaches adult levels by

Answer 28

36 weeks

Question 29

Intermediate phase of acute bilirubin encephalopathy

Answer 29

exchange transfusion

Question 30

exchange transfusion removes _____% of neonate's RBCs

Answer 30

85%

Question 31

What causes purpura fulminans

Answer 31

absence of protein C or S

Question 32

Hemorrhagic dz of newborn vs hemophilia coags

Answer 32

Hemorrhagic: ISOLATED PT, normal PTT and platelets Hemophilia = isolated prolongation of PTT, normal everything else

Question 33

Methemoglobinemia cause and tx

Answer 33

cytochrome b5 reductase If level > 40%, tx with methylene blue

Question 34

Staph scalded skin syndrome has gram stain of ____ from bullae

Answer 34

sterile culture

Question 35

Pyloric atresia + multiple blisters

Answer 35

epidermolysis bulls (Herlitz and Carmi syndrome)

Question 36

Do we fix dolicocephaly? (Premature closure of sagittal suture)

Answer 36

yes, for cosmetics. no intracranial or neurologic problems

Question 37

After MMC repair most kids can

Answer 37

ambulate with or without short braces

Question 38

Selective neuronal necrosis

Answer 38

most common pattern of cerebral injury after HIE —> mental deficiency, seizures, ataxia, feeding difficulties, pyramidal cerebral palsy

Question 39

Extrapyramidal or athetoid/dyskinetic cerebral palsy

Answer 39

mixed tone in same muscle with gross and fine motor skills affected. Hearing and speech problems exist Other types: Spastic (hemiegia, diplegia, quadriplegia) Ataxic - affects balance and depth perception Mixed

Question 40

McCune Albright Syndrome

Answer 40

multiple areas of fibrous dysplasia of long bones but also ribs and spine. Irregular brown pigmentation on sacrum buttocks, upper spine. Can also have hyperthyroidism, hyperparathyroidism, pituitary adenomas

Question 41

Biphasic stridor is result of

Answer 41

laryngeal obstruction (laryngomalacia)

Question 42

Fetal mortality rate is

Answer 42

Fetal deaths > 28 weeks divided by number of live births and fetal deaths >28 weeks x 1000

Question 43

Ordinal / ranked, categorical/nominal, discrete

Answer 43

Sarnat = ordinal / ranked(beginner, medium, expert). There are ranks. Categorical / Nominal= like ordinal, but no ranks. (College majors) Discrete = data can take certain values usually whole numbers. (Number of students in class)

Question 44

Weakness of case control is

Answer 44

recall bias

Question 45

What hormone from placenta initiates genital differentiation

Answer 45

HcG 12 weeks, fetus produces GnRH; and LH and FSH stimulates fetal gonads to continue with genital development Hypothalamus provides GnRH

Question 46

Crossed adductor sign present until ____ Moro/palmar/galant disappear by ____

Answer 46

7-8 months 6 months

Question 47

pupillary light response is variably present by ____ weeks, definitely present by ____ weeks

Answer 47

30, 34

Question 48

Most common cystic renal dz in newborn period

Answer 48

multi-cystic dysplastic kidneys