childhood

Question 1

agenesis

Answer 1

absent organ, absent primordial tissue

Question 2

aplasia

Answer 2

absent organ, primodrial tissue present

Question 3

hypoplasia

Answer 3

incomplete organ development

Question 4

how infant is exposed to transcervical perinatal infection? disease caused

Answer 4

“inhale” in utero or via birth process; common cause of pneumonia

Question 5

how infant is exposed to trans placental perinatal infection? disease causes

Answer 5

via blood through chorionic villi, TORCH infections

Question 6

what is the underlying cause of RDS?

Answer 6

deficient surfactant

Question 7

what are the risk factors for RDS?

Answer 7

male, maternal DM, prematurity, C-section

Question 8

why is maternal DM a risk for RDS?

Answer 8

high insulin levels suppress surfactant production

Question 9

why is C-section a risk for RDS?

Answer 9

decrease in stress-induced steroid release leads to less surfactant

Question 10

what composes the hyaline membranes?

Answer 10

necrotic epithelial cells and plasma proteins

Question 11

2 complications associated with O2 therapy for RDS

Answer 11

retinopathy of prematurity

bronchopulmonary dysplasia/dec septation

Question 12

what are the 2 phases of retinopathy of prematurity?

Answer 12

1- decreased VEGF/apoptosis

2- increased VEGF/neovascularization

Question 13

what is the typical scenario for NEC?

Answer 13

premature infant who just started enteral feeds

Question 14

what is the characteristic Xray finding in NEC?

Answer 14

submucosal gas bubbles (pneumatosis intestinalis)

Question 15

3 presenting symptoms of NEC

Answer 15

bloody stool, abdominal distention, circulatory collapse

Question 16

definition of SIDS

Answer 16

sudden, unexpected death of infant under 1 that remains unexplained after autopsy, review of clinical hx and investigation of scene

Question 17

90% of SIDS cases occur between what ages

Answer 17

2-4 mo

Question 18

risk factors for SIDS (11)

Answer 18

young mom, smoking, male, premature, multiple, short time between babies, parental drug use, family hx SIDS, hyperthermia, soft surface, prone position

Question 19

Rh immune hydrops is due to

Answer 19

Rh incompatibility (Rh- mon, Rh+ baby)

Question 20

infant with immune hydrops will have what additional features?

Answer 20

jaundice and kernicterus (due to hemolysis)

Question 21

which antigen is the focus of immune hydrops?

Answer 21

D antigen

Question 22

why is ABO incompatibility now a more common cause of immune hydrops?

Answer 22

effective treatment of Rh with RHOgam

Question 23

what is the mother/baby problem with ABO incompatibility?

Answer 23

O type mother, A or B type fetus (may have anti-A or B IgG that can cross placenta)

Question 24

immune hydrops sensitization for Rh vs ABO

Answer 24

Rh- requires

ABO- does not require, can’t affect first pregnancy

Question 25

most common infection to cause hydrops

Answer 25

parvo B19

Question 26

most common fetal anemia to cause hydrous

Answer 26

alpha thalassemia (Hb barts)

Question 27

most common benign tumor of infancy

Answer 27

hemangioma

Question 28

course for hemangiomas

Answer 28

may enlarge as child gets older, but most spontaneously regress

Question 29

most common germ cell tumor of children

Answer 29

teratoma

Question 30

neuroblastomas are composed of-

Answer 30

postganglionic sympathetic neurons

Question 31

main location of neuroblastoma

Answer 31

adrenal medulla

Question 32

labs in neuroblastoma

Answer 32

elevated urine VMA, HVA

Question 33

genetic abnormality of neuroblastoma

Answer 33

amplification of N-myc

Question 34

prognosis is based on/exception

Answer 34

based on stage (higher=worse), except for 4S (infants under 1)

Question 35

mutation assoc with retinoblastoma

Answer 35

point mutation on chromosome 13 that inactivates RB supressor gene

Question 36

patients with germ line mutation are at increased risk for-

Answer 36

osteosarcoma later in life

Question 37

why is retinoic acid teratogenic? (2)

Answer 37

down-regulation of TGFb | interferes with HOX

Question 38

ureteroplacental insufficiency will affect growth but spares the-

Answer 38

head/brain

Question 39

classic triad of congenital rubella

Answer 39

PDA, cataracts, blueberry muffin rash

Question 40

fetal alcohol syndrome presentation (4)

Answer 40

elongated philtrim, frontal bossing, heart murmur, eye problems

Question 41

first test to do if women has 3+ spontaneous abortions, followed by

Answer 41

chromosomal analysis (followed by testing for maternal antibodies/coagulopathy)

Question 42

effect of maternal DM on fetal pancreas

Answer 42

islet hyperplasia

Question 43

cells that make sufactant

Answer 43

type II pneumocytes

Question 44

L:S ratio that indicates lung maturity

Answer 44

greater than 2:1

Question 45

PKU is caused by deficiency of

Answer 45

phenylalanine hydroxylase

Question 46

presumed precursor to wilms tumor

Answer 46

nephrogenic "rests"

Question 47

NT involved in SIDS

Answer 47

serotonin (respiratory center in the medulla)