childhood Flashcards

1
Q

agenesis

A

absent organ, absent primordial tissue

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2
Q

aplasia

A

absent organ, primodrial tissue present

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3
Q

hypoplasia

A

incomplete organ development

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4
Q

how infant is exposed to transcervical perinatal infection? disease caused

A

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

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5
Q

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

A

via blood through chorionic villi, TORCH infections

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6
Q

what is the underlying cause of RDS?

A

deficient surfactant

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7
Q

what are the risk factors for RDS?

A

male, maternal DM, prematurity, C-section

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8
Q

why is maternal DM a risk for RDS?

A

high insulin levels suppress surfactant production

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9
Q

why is C-section a risk for RDS?

A

decrease in stress-induced steroid release leads to less surfactant

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10
Q

what composes the hyaline membranes?

A

necrotic epithelial cells and plasma proteins

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11
Q

2 complications associated with O2 therapy for RDS

A

retinopathy of prematurity

bronchopulmonary dysplasia/dec septation

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12
Q

what are the 2 phases of retinopathy of prematurity?

A

1- decreased VEGF/apoptosis

2- increased VEGF/neovascularization

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13
Q

what is the typical scenario for NEC?

A

premature infant who just started enteral feeds

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14
Q

what is the characteristic Xray finding in NEC?

A

submucosal gas bubbles (pneumatosis intestinalis)

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15
Q

3 presenting symptoms of NEC

A

bloody stool, abdominal distention, circulatory collapse

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16
Q

definition of SIDS

A

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

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17
Q

90% of SIDS cases occur between what ages

A

2-4 mo

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18
Q

risk factors for SIDS (11)

A

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

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19
Q

Rh immune hydrops is due to

A

Rh incompatibility (Rh- mon, Rh+ baby)

20
Q

infant with immune hydrops will have what additional features?

A

jaundice and kernicterus (due to hemolysis)

21
Q

which antigen is the focus of immune hydrops?

A

D antigen

22
Q

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

A

effective treatment of Rh with RHOgam

23
Q

what is the mother/baby problem with ABO incompatibility?

A

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

24
Q

immune hydrops sensitization for Rh vs ABO

A

Rh- requires

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

25
Q

most common infection to cause hydrops

A

parvo B19

26
Q

most common fetal anemia to cause hydrous

A

alpha thalassemia (Hb barts)

27
Q

most common benign tumor of infancy

A

hemangioma

28
Q

course for hemangiomas

A

may enlarge as child gets older, but most spontaneously regress

29
Q

most common germ cell tumor of children

A

teratoma

30
Q

neuroblastomas are composed of-

A

postganglionic sympathetic neurons

31
Q

main location of neuroblastoma

A

adrenal medulla

32
Q

labs in neuroblastoma

A

elevated urine VMA, HVA

33
Q

genetic abnormality of neuroblastoma

A

amplification of N-myc

34
Q

prognosis is based on/exception

A

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

35
Q

mutation assoc with retinoblastoma

A

point mutation on chromosome 13 that inactivates RB supressor gene

36
Q

patients with germ line mutation are at increased risk for-

A

osteosarcoma later in life

37
Q

why is retinoic acid teratogenic? (2)

A

down-regulation of TGFb

interferes with HOX

38
Q

ureteroplacental insufficiency will affect growth but spares the-

A

head/brain

39
Q

classic triad of congenital rubella

A

PDA, cataracts, blueberry muffin rash

40
Q

fetal alcohol syndrome presentation (4)

A

elongated philtrim, frontal bossing, heart murmur, eye problems

41
Q

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

A

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

42
Q

effect of maternal DM on fetal pancreas

A

islet hyperplasia

43
Q

cells that make sufactant

A

type II pneumocytes

44
Q

L:S ratio that indicates lung maturity

A

greater than 2:1

45
Q

PKU is caused by deficiency of

A

phenylalanine hydroxylase

46
Q

presumed precursor to wilms tumor

A

nephrogenic “rests”

47
Q

NT involved in SIDS

A

serotonin (respiratory center in the medulla)