Pediatric Board Review Questions Flashcards
Central cyanosis occurs when
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
Cause of prolonged QT interval
hypocalcemia
Hypercalcemia conversely causes shortened QT
Oxygen delivery to alveoli
(ml/kg/min) = alveolar MV x FiO2
Alveolar MV = TV x RR
Increasing CO will change O2 delivery but not content
reminder is all
Incidence of Single UA
1%. More common in twins (3-4x)
Pre-E vs severe pre-E
Pre-E: SBP>140, DBP > 90
Severe is SBP>160, DBP >110
T18 quad screen
low / low / low / normal = T18
Teratogenic effects of isotretinoin
TGA, triangular face, microtia, hypertelorism, epicentral folds, small arms, HC > 95%
Intrauterine hypoxia favors which organs
heart, brain, adrenals
Blood returning from placenta to fetus has PaO2 of
20-30
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
1/3; 45%
Cyanosis, single heart sound, L superior axis on ECG
Tricuspid atresia
Hunter syndrome inheritance
x-linked; Hurler, Sanfillipo, Morquio, Sly are AR
Crouzon syndrome
proptosis, maxillary hypoplasia, craniosynostosis
Cat-eye syndrome
anal atresia, coloboma of iris, extra part of chromosome 22, TAPVR
Meckel Gruber syndrome
Polydactyly, cystic dysplastic kidneys, encephalocele
What are essential AA
Methionine, valine, leucine, threonine
Arginine is essential in preterm
highest fatty acid concentration in mature BM (which one)
oleic acid
Human colostrum compared to mature BM
more protein
in CAH, aldosterone is
low
Na deficit equation
[na desired - na current][0.6][weight]
Profound metabolic acidosis, slightly elevated ammonia
organic acidemia
Severely elevated ammonia, respiratory alkalosis
urea cycle
Zelwegger syndrome
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
Chronic granulomatous disease and dx test
Chronic granulomatous dz = recurrent infections with catalase + bacteria or fungi
Nitroblue tetrazolium test or dihydrorhodamine 123 test
SCID presentation
diarrhea, FTT, scaly eruption combined with EOSINOPHILIA
Tx for infants born to mothers with gonorrhea
single IV CTX
Lactase reaches adult levels by
36 weeks
Intermediate phase of acute bilirubin encephalopathy
exchange transfusion
exchange transfusion removes _____% of neonate’s RBCs
85%
What causes purpura fulminans
absence of protein C or S
Hemorrhagic dz of newborn vs hemophilia coags
Hemorrhagic: ISOLATED PT, normal PTT and platelets
Hemophilia = isolated prolongation of PTT, normal everything else
Methemoglobinemia cause and tx
cytochrome b5 reductase
If level > 40%, tx with methylene blue
Staph scalded skin syndrome has gram stain of ____ from bullae
sterile culture
Pyloric atresia + multiple blisters
epidermolysis bulls (Herlitz and Carmi syndrome)
Do we fix dolicocephaly? (Premature closure of sagittal suture)
yes, for cosmetics. no intracranial or neurologic problems
After MMC repair most kids can
ambulate with or without short braces
Selective neuronal necrosis
most common pattern of cerebral injury after HIE —> mental deficiency, seizures, ataxia, feeding difficulties, pyramidal cerebral palsy
Extrapyramidal or athetoid/dyskinetic cerebral palsy
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
McCune Albright Syndrome
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
Biphasic stridor is result of
laryngeal obstruction (laryngomalacia)
Fetal mortality rate is
Fetal deaths > 28 weeks divided by number of live births and fetal deaths >28 weeks x 1000
Ordinal / ranked, categorical/nominal, discrete
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)
Weakness of case control is
recall bias
What hormone from placenta initiates genital differentiation
HcG
12 weeks, fetus produces GnRH; and LH and FSH stimulates fetal gonads to continue with genital development
Hypothalamus provides GnRH
Crossed adductor sign present until ____
Moro/palmar/galant disappear by ____
7-8 months
6 months
pupillary light response is variably present by ____ weeks, definitely present by ____ weeks
30, 34
Most common cystic renal dz in newborn period
multi-cystic dysplastic kidneys